J Jacc 2022 08 004

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. -, NO.
-, 2022
ª 2022 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION
AND THE AMERICAN HEART ASSOCIATION, INC.
PUBLISHED BY ELSEVIER
CLINICAL PRACTICE GUIDELINE
2022 ACC/AHA Guideline for the

Diagnosis and Management of
Aortic Disease
A Report of the American Heart Association/American College of Cardiology
Joint Committee on Clinical Practice Guidelines
Developed in collaboration with and endorsed by the American Association for Thoracic Surgery,
American College of Radiology, Society of Cardiovascular Anesthesiologists,
Society for Cardiovascular Angiography and Interventions, Society of Thoracic Surgeons,
and Society for Vascular Surgery
Endorsed by the Society of Interventional Radiology and Society for Vascular Medicine
Writing Eric M. Isselbacher, MD, MSC, FACC, Chair Dianna M. Milewicz, MD, PHD
Committee Ourania Preventza, MD, MBA, Vice Chair Gustavo S. Oderich, MD
Members* James Hamilton Black III, MD, DFSVS, Vice Chair Laura Ogbechie, MSN
Susan B. Promes, MD, MBA
John G. Augoustides, MD, FAHAy Elsie Gyang Ross, MD, MSC
Adam W. Beck, MD, DFSVS Marc L. Schermerhorn, MD, DFSVSxx
Michael A. Bolen, MDz Sabrina Singleton Times, DHSC, MPHjjjj
Alan C. Braverman, MD, FACC Elaine E. Tseng, MD, FAHA
Bruce E. Bray, MD, FACCx Grace J. Wang, MD, MSCE
Maya M. Brown-Zimmerman, MPHjj Y. Joseph Woo, MD, FACC, FAHAyy
Edward P. Chen, MD, FAHA
Tyrone J. Collins, MD, MSCAI, FACC, FAHA, FSVM{
*Writing committee members are required to recuse themselves from
Abe DeAnda JR, MD, FAHA
voting on sections to which their specific relationships with industry may
Christina L. Fanola, MD, MSC apply; see Appendix 1 for detailed information. ySCA representative. zACR
#
Leonard N. Girardi, MD, FAHA representative. xAHA/ACC Joint Committee on Clinical Data Standards
Caitlin W. Hicks, MD, MS, FSVS** liaison. jjLay stakeholder representative. {SCAI representative. #AATS
representative. **ACC/AHA Joint Committee on Performance Measures
Dawn S. Hui, MD
liaison. yyAHA/ACC Joint Committee on Clinical Practice Guidelines
William Schuyler Jones, MD, FACCyy liaison. zzSTS representative. xxSVS representative. jjjjAHA/ACC staff
Vidyasagar Kalahasti, MD, FACC representative.
Karen M. Kim, MD, MSzz
This document was approved by the American College of Cardiology Clinical Policy Approval Committee and the American Heart Association Science
Advisory and Coordinating Committee in July 2022, the American College of Cardiology Science and Quality Committee in August 2022, and the
American Heart Association Executive Committee in September 2022.
The American College of Cardiology requests that this document be cited as follows: Isselbacher EM, Preventza O, Black JH 3rd, Augoustides JG, Beck
AW, Bolen MA, Braverman AC, Bray BE, Brown-Zimmerman MM, Chen EP, Collins TJ, DeAnda A Jr, Fanola CL, Girardi LN, Hicks CW, Hui DS, Jones WS,
Kalahasti V, Kim KM, Milewicz DM, Oderich GS, Ogbechie L, Promes SB, Ross EG, Schermerhorn ML, Times SS, Tseng EE, Wang GJ, Woo YJ. 2022 ACC/
AHA guideline for the diagnosis and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint
Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022;xx:xxx-xxx.
This article has been copublished in Circulation.
Copies: This document is available on the websites of the American College of Cardiology (www.acc.org) and the American Heart Association
(professional.heart.org). For copies of this document, please contact the Elsevier Inc. Reprint Department via fax (212-633-3820) or e-mail (reprints@
elsevier.com).
Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not permitted without the express
permission of the American College of Cardiology. Requests may be completed online via the Elsevier site (http://www.elsevier.com/about/policies/
author-agreement/obtaining-permission).
ISSN 0735-1097/$36.00 https://doi.org/10.1016/j.jacc.2022.08.004

2 Isselbacher et al JACC VOL. -, NO. -, 2022
2022 ACC/AHA Aortic Disease Guideline -, 2022:-–-
Peer David P. Faxon, MD, FACC, Chair S. Christopher Malaisrie, MD, FACC#
Review Gilbert R. Upchurch JR, MD, FAHA, FACC, Vice Chair Kathryn Osteen, PHD, RN, CMSRN, CNE
Committee Himanshu J. Patel, MD, FACC, FAHA
Members Aaron W. Aday, MD, MSC, FAHA Parag J. Patel, MD{{
Ali Azizzadeh, MD Wanda M. Popescu, MD
Michael Boisen, MDy Evelio Rodriguez, MD, FACC
Beau Hawkins, MD, FACC{ Rebecca Sorber, MD
Christopher M. Kramer, MD, FACC, FAHA Philip S. Tsao, PHD
Jessica G.Y. Luc, MD Annabelle Santos Volgman, MD, FACC, FAHA
Thomas E. MacGillivray, MDzz
{{SIR representative.
AHA/ACC Joint Joshua A. Beckman, MD, MS, FAHA, FACC, Chair Adrian F. Hernandez, MD, MHS, FAHA
Committee Catherine M. Otto, MD, FAHA, FACC, Chair-Elect José A. Joglar, MD, FACC, FAHA##
Members Patrick T. O’Gara, MD, MACC, FAHA, Immediate Past W. Schuyler Jones, MD, FACC
Chair## Daniel Mark, MD, MPH, FACC, FAHA##
Debabrata Mukherjee, MD, FACC, FAHA, FSCAI
Anastasia Armbruster, PHARMD, FACC Latha Palaniappan, MD, MS, FACC, FAHA
Kim K. Birtcher, PHARMD, MS, FACC## Mariann R. Piano, RN, PHD, FAHA
Lisa de las Fuentes, MD, MS, FAHA Tanveer Rab, MD, FACC
Anita Deswal, MD, MPH, FACC, FAHA Erica S. Spatz, MD, MS, FACC
Dave L. Dixon, PHARMD, FACC, FAHA## Jacqueline E. Tamis-Holland, MD, FAHA, FACC, FSCAI
Bulent Gorenek, MD, FACC Y. Joseph Woo, MD, FACC, FAHA
Norrisa Haynes, MD, MPH ##Former Joint Committee on Clinical Practice Guideline member,
current member during the writing effort.
ABSTRACT
AIM The “2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease” provides recommendations to guide cli-
nicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-
term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symp-
tomatic, and acute aortic syndromes).
METHODS A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and
other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL
Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during
the guideline writing process, were also considered by the writing committee, where appropriate.
STRUCTURE Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery dis-
ease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations
addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared
decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an
increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of
patients with aortic disease.
CONTENTS
ABSTRACT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . - 1.2. Organization of the Writing Committee . . . . . . . . -
1.3. Document Review and Approval . . . . . . . . . . . . . . -

TOP 10 TAKE-HOME MESSAGES . . . . . . . . . . . . . . . . . . . -
1.4. Scope of the Guideline . . . . . . . . . . . . . . . . . . . . . . -
PREAMBLE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -
1.5. Class of Recommendations and
1. INTRODUCTION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . - Level of Evidence . . . . . . . . . . . . . . . . . . . . . . . . . . -
1.1. Methodology and Evidence Review . . . . . . . . . . . - 1.6. Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -

JACC VOL. -, NO. -, 2022 Isselbacher et al 3
-, 2022:-–- 2022 ACC/AHA Aortic Disease Guideline
2. NORMAL ANATOMY, ABNORMAL ANATOMY, 6.5.5. Abdominal Aortic Aneurysms . . . . . . . . . . . -

AND DEFINITIONS . . . . . . . . . . . . . . . . . . . . . . . . . . . . - 6.5.6. Surveillance After Aneurysm Repair . . . . . -
2.1. Normal Aortic Anatomy . . . . . . . . . . . . . . . . . . . . . - 7. ACUTE AORTIC SYNDROMES . . . . . . . . . . . . . . . . . . -
2.2. Aortic Landing Zones . . . . . . . . . . . . . . . . . . . . . . . - 7.1. Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -
2.3. Definitions of Dilation and Aneurysm of the 7.2. AAS: Diagnostic Evaluation (Imaging,
Aortic Root and Ascending Thoracic Aorta . . . . . . - Laboratory Testing) . . . . . . . . . . . . . . . . . . . . . . . . -
2.3.1. Normalizing Aortic Root and Ascending 7.3. Medical Management of AAS . . . . . . . . . . . . . . . . . -
Aortic Diameters for Body Size . . . . . . . . . . -
7.3.1. Acute Medical Management of AAS . . . . . . -
2.4. Definitions and Classification of Acute Aortic 7.3.2. Subsequent Medical Management of AAS . -
Syndrome (AAS) . . . . . . . . . . . . . . . . . . . . . . . . . . . -
7.4. Surgical and Endovascular Management of
2.5. Classification of Thoracoabdominal Aortic
Acute Aortic Dissection . . . . . . . . . . . . . . . . . . . . . -
Aneurysm (TAAA) . . . . . . . . . . . . . . . . . . . . . . . . . . -
7.4.1. Acute Type A Aortic Dissection . . . . . . . . . . -
2.6. Classification of Endoleaks . . . . . . . . . . . . . . . . . . -
7.4.2. Management of Acute Type B
Aortic Dissection . . . . . . . . . . . . . . . . . . . . . -
3. IMAGING AND MEASUREMENTS . . . . . . . . . . . . . . . . -
3.1. Aortic Imaging Techniques to Determine 7.5. Management of IMH . . . . . . . . . . . . . . . . . . . . . . . -
Presence and Progression of Aortic Disease . . . . . -

7.6. Management of PAU . . . . . . . . . . . . . . . . . . . . . . . . -
3.2. Conventions of Measurements . . . . . . . . . . . . . . . - 7.6.1. PAU With IMH, Rupture, or Both . . . . . . . . -
3.2.1. Computed Tomography . . . . . . . . . . . . . . . . . - 7.6.2. Isolated PAU . . . . . . . . . . . . . . . . . . . . . . . . . -
3.2.2. Magnetic Resonance Imaging . . . . . . . . . . . . - 7.6.3. PAU Open Surgical Repair Versus
3.2.3. Echocardiography . . . . . . . . . . . . . . . . . . . . . . - Endovascular Repair . . . . . . . . . . . . . . . . . . -
3.2.4. Intravascular Ultrasound . . . . . . . . . . . . . . . . - 7.7. Traumatic Aortic Injury . . . . . . . . . . . . . . . . . . . . . -
3.2.5. Abdominal Ultrasound . . . . . . . . . . . . . . . . . . - 7.7.1. Initial Management of Blunt Traumatic

Thoracic Aortic Injury (BTTAI) . . . . . . . . . . -
4. MULTIDISCIPLINARY AORTIC TEAMS . . . . . . . . . . . -
7.7.2. Initial Management of Blunt Traumatic
5. SHARED DECISION-MAKING . . . . . . . . . . . . . . . . . . . -
Abdominal Aortic Injury (BAAI) . . . . . . . . . -
7.7.3. Long-Term Management and Surveillance

6. ANEURYSMS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . - After Blunt Traumatic Aortic Injury
(BTAI) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -
6.1. Thoracic Aortic Aneurysm (TAA) Causes . . . . . . . -
6.1.1. Sporadic and Degenerative TAA . . . . . . . . . - 7.8. Long-Term Management and Surveillance
Imaging After AAS . . . . . . . . . . . . . . . . . . . . . . . . . -
6.1.2. Genetic Aortopathies . . . . . . . . . . . . . . . . . . -
7.8.1. Long-Term Surveillance Imaging After
6.1.3. BAV Aortopathy . . . . . . . . . . . . . . . . . . . . . . -
Aortic Dissection and IMH . . . . . . . . . . . . . . -
6.2. AAA: Cause, Risk Factors, and Screening . . . . . . . - 7.8.2. Long-Term Management After Acute
Aortic Dissection and IMH . . . . . . . . . . . . . . -
6.3. Growth and Natural History of
Aortic Aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . - 7.8.3. Long-Term Management and
Surveillance for PAUs . . . . . . . . . . . . . . . . . -
6.4. Medical Management of Sporadic and
Degenerative Aortic Aneurysm Disease . . . . . . . . - 8. PREGNANCY IN PATIENTS WITH AORTOPATHY . . -
6.4.1. Medical Therapy and Risk Factor 8.1. Counseling and Management of Aortic Disease in
Modification in Sporadic TAA . . . . . . . . . . . - Pregnancy and Postpartum . . . . . . . . . . . . . . . . . . -
6.4.2. Medical Therapy and Risk Factor 8.2. Delivery in Pregnant Patients With Aortopathy . . -
Modification in AAA . . . . . . . . . . . . . . . . . . -
6.4.3. Surveillance for Medical Management . . . . -

8.3. Surgery Before Pregnancy in Women With
Aortic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -
6.5. Surgical and Endovascular Management of
8.4. Pregnancy in Patients With Aortopathy: Aortic
Aortic Aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . -
Dissection and Aortic Surgery in Pregnancy . . . . -
6.5.1. Surgery for Sporadic Aneurysms of the
Aortic Root and Ascending Aorta . . . . . . . . -
9. OTHER AORTIC CONDITIONS . . . . . . . . . . . . . . . . . . -
6.5.2. Aortic Arch Aneurysms . . . . . . . . . . . . . . . . -
9.1. Inflammatory Aortitis: Diagnosis and
6.5.3. Descending TAA . . . . . . . . . . . . . . . . . . . . . . -
Treatment of Takayasu Arteritis and
6.5.4. Thoracoabdominal Aortic Aneurysms . . . . - Giant Cell Arteritis (GCA) . . . . . . . . . . . . . . . . . . . . -
9.2. Infectious Aortitis . . . . . . . . . . . . . . . . . . . . . . . . . . - capabilities, Multidisciplinary Aortic Team care is

9.2.1. Diagnosis and Management of considered in determining the appropriate timing of
Infection of the Native Aorta . . . . . . . . . . . - intervention.
9.2.2. Diagnosis and Management of 2. Shared decision-making involving the patient and a
Prosthetic Aortic Graft Infection . . . . . . . . . - multidisciplinary team is highly encouraged to
9.3. Atherosclerotic Disease . . . . . . . . . . . . . . . . . . . . . -
determine the optimal medical, endovascular, and
open surgical therapies. In patients with aortic disease
9.3.1. Aortic Thrombus . . . . . . . . . . . . . . . . . . . . . -
who are contemplating pregnancy or who are preg-
9.3.2. Aortic Occlusion . . . . . . . . . . . . . . . . . . . . . . -
nant, shared decision-making is especially important
9.3.3. Porcelain Aorta . . . . . . . . . . . . . . . . . . . . . . . -
when considering the cardiovascular risks of preg-
9.4. Coarctation of the Aorta (CoA) and nancy, the diameter thresholds for prophylactic aortic
Congenital Abnormalities of the Arch . . . . . . . . . . -
surgery, and the mode of delivery.
9.4.1. Coarctation of the Aorta . . . . . . . . . . . . . . . - 3. Computed tomography, magnetic resonance imaging,
9.4.2. Other Arch Abnormalities . . . . . . . . . . . . . . - and echocardiographic imaging of patients with aortic
disease should follow recommended approaches for
9.5. Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -
image acquisition, measurement and reporting of
relevant aortic dimensions, and the frequency of
10. PHYSICAL ACTIVITY AND QUALITY OF LIFE . . . . . -
surveillance before and after intervention.
4. At centers with Multidisciplinary Aortic Teams and
11. COST AND VALUE CONSIDERATIONS . . . . . . . . . . . -
experienced surgeons, the threshold for surgical
intervention for sporadic aortic root and ascending
12. EVIDENCE GAPS AND FUTURE DIRECTIONS . . . . . -
aortic aneurysms has been lowered from 5.5 cm to 5.0
12.1. Biomarker Studies . . . . . . . . . . . . . . . . . . . . . . . . . - cm in selected patients, and even lower in specific
12.2. Genetic and Nongenetic Factors . . . . . . . . . . . . . -
scenarios among patients with heritable thoracic
aortic aneurysms.
12.3. Biomechanics of the Aorta . . . . . . . . . . . . . . . . . . -
5. In patients who are significantly smaller or taller than
12.4. Sex, Race, and Ethnicity . . . . . . . . . . . . . . . . . . . . - average, surgical thresholds may incorporate indexing
of the aortic root or ascending aortic diameter to
12.5. Quality of Life in Patients With Aortic Disease . . -
either patient body surface area or height, or aortic
12.6. New Endovascular Technology . . . . . . . . . . . . . . - cross-sectional area to patient height.
12.7. Optimal Exercise and Rehabilitation Protocols . . - 6. Rapid aortic root growth or ascending aortic aneurysm
growth, an indication for intervention, is defined
12.8. Equitable Care and Training Opportunities . . . . . -
as $0.5 cm in 1 year or $0.3 cm per year in 2 consec-
utive years for those with sporadic aneurysms
REFERENCES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -
and $0.3 cm in 1 year for those with heritable thoracic
aortic disease or bicuspid aortic valve.
APPENDIX 1
7. In patients undergoing aortic root replacement sur-
Author Relationships With Industry and Other gery, valve-sparing aortic root replacement is
Entities (Relevant) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . - reasonable if the valve is suitable for repair and when
performed by experienced surgeons in a Multidisci-
APPENDIX 2 plinary Aortic Team.
Reviewer Relationships With Industry and Other 8. Patients with acute type A aortic dissection, if clini-
Entities (Comprehensive) . . . . . . . . . . . . . . . . . . . . . . . - cally stable, should be considered for transfer to a
high-volume aortic center to improve survival. The
operative repair of type A aortic dissection should
TOP 10 TAKE-HOME MESSAGES FOR
entail at least an open distal anastomosis rather than
THE DIAGNOSIS AND MANAGEMENT OF
just a simple supracoronary interposition graft.
AORTIC DISEASE
9. There is an increasing role for thoracic endovascular
1. Because outcomes for patients with aortic disease are aortic repair in the management of uncomplicated
enhanced at programs with higher volumes, experi- type B aortic dissection. Clinical trials of repair of
enced practitioners, and extensive management thoracoabdominal aortic aneurysms with endografts
are reporting results that suggest endovascular repair updates, and modifies guideline methodology on
is an option for patients with suitable anatomy. the basis of published standards from organizations,
10. In patients with aneurysms of the aortic root or including the Institute of Medicine, 1,2 and on the
ascending aorta, or those with aortic dissection, basis of internal reevaluation. Similarly, presentation
screening of first-degree relatives with aortic imaging and delivery of guidelines are reevaluated and
is recommended. modified in response to evolving technologies and
other factors to optimally facilitate dissemination of
PREAMBLE information to health care professionals at the point
of care.
Since 1980, the American College of Cardiology (ACC) Numerous modifications to the guidelines have
and American Heart Association (AHA) have translated been implemented to make them shorter and enhance
scientific evidence into clinical practice guidelines with “user friendliness.” Guidelines are written and pre-
recommendations to improve cardiovascular health. sented in a modular, “knowledge chunk” format, in
These guidelines, which are based on systematic methods which each chunk includes a table of recommenda-
to evaluate and classify evidence, provide a foundation tions, a brief synopsis, recommendation-specific sup-
for the delivery of quality cardiovascular care. The ACC portive text and, when appropriate, flow diagrams or
and AHA sponsor the development and publication of additional tables. Hyperlinked references are provided
clinical practice guidelines without commercial support, for each modular knowledge chunk to facilitate quick
and members volunteer their time to the writing and access and review.
review efforts. Guidelines are official policy of the In recognition of the importance of cost–value consid-
ACC and AHA. For some guidelines, the ACC and AHA erations, in certain guidelines, when appropriate and
collaborate with other organizations. feasible, an analysis of value for a drug, device, or inter-
vention may be performed in accordance with the ACC/
Intended Use AHA methodology.3
Clinical practice guidelines provide recommendations To ensure that guideline recommendations remain
applicable to patients with or at risk of developing car- current, new data will be reviewed on an ongoing basis by
diovascular disease. The focus is on medical practice in the writing committee and staff. Going forward, targeted
the United States, but these guidelines are relevant to sections/knowledge chunks will be revised dynamically
patients throughout the world. Although guidelines may after publication and timely peer review of potentially
be used to inform regulatory or payer decisions, the intent practice-changing science. The previous designations of
is to improve quality of care and align with patients’ in- “full revision” and “focused update” will be phased out.
terests. Guidelines are intended to define practices For additional information and policies on guideline
meeting the needs of patients in most, but not all, cir- development, readers may consult the ACC/AHA guide-
cumstances and should not replace clinical judgment. line methodology manual4 and other methodology arti-
cles.5-7
Clinical Implementation
Management, in accordance with guideline recommen- Selection of Writing Committee Members
dations, is effective only when followed by both practi- The Joint Committee strives to ensure that the guide-
tioners and patients. Adherence to recommendations can line writing committee contains requisite content
be enhanced by shared decision-making between clini- expertise and is representative of the broader cardio-
cians and patients, with patient engagement in selecting vascular community by selection of experts across a
interventions on the basis of individual values, prefer- spectrum of backgrounds, representing different
ences, and associated conditions and comorbidities. geographic regions, sexes, races, ethnicities, intellectual
perspectives/biases, and clinical practice settings. Or-
Methodology and Modernization ganizations and professional societies with related in-
The AHA/ACC Joint Committee on Clinical Practice terests and expertise are invited to participate as
Guidelines (Joint Committee) continuously reviews, partners or collaborators.
Relationships With Industry and Other Entities 1. INTRODUCTION

The ACC and AHA have rigorous policies and methods to
ensure that documents are developed without bias or 1.1. Methodology and Evidence Review
improper influence. The complete policy on relationships The recommendations listed in this guideline are,
with industry and other entities (RWI) can be found on- whenever possible, evidence based. An initial extensive
line. Appendix 1 of the guideline lists writing committee evidence review, which included literature derived
members’ relevant RWI. For the purposes of full trans- from research involving human subjects, published in
parency, their comprehensive disclosure information is English, and indexed in MEDLINE (through PubMed),
available in a Supplemental Appendix. Comprehensive EMBASE, the Cochrane Library, the Agency for Healthcare
disclosure information for the Joint Committee is also Research and Quality, and other selected databases
available online. relevant to this guideline, was conducted from February
2021 to April 2021. Search terms included both key words
Evidence Review and Evidence Review Committees and index terms (eg, MeSH, Emtree); search terms
In developing recommendations, the writing committee included but were not limited to the following: aortic
uses evidence-based methodologies that are based on all occlusion; aortic aneurysm; aortic aneurysm, thoracic;
available data. 4,5
Literature searches focus on randomized aortic aneurysm, abdominal; surveillance after endovas-
controlled trials (RCTs) but also include registries, non- cular aneurysm repair; diagnostic imaging; monitoring;
randomized comparative and descriptive studies, case surveillance; imaging; aorta; aortic; computed tomography;
series, cohort studies, systematic reviews, and expert ultrasound; magnetic resonance imaging; arterial occlusive
opinion. Only key references are cited. diseases; aortic diseases; aortic atherosclerosis; athero-
An independent evidence review committee is sclerosis; clinical trial; observational study; randomized
commissioned when there are $1 question(s) deemed of controlled trial; review; atherosclerotic aortic disease;
utmost clinical importance and merit formal systematic plaque, atherosclerotic; aorta; aortitis; infectious; autoim-
review to determine which patients are most likely to mune; aortic rupture; penetrating aortic ulcers; compara-
benefit from a drug, device, or treatment strategy, and to tive studies; nonexperimental studies; type A aortic
what degree. Criteria for commissioning an evidence re- dissection; type A; type B; aneurysm, dissecting; aorta and
view committee and formal systematic review include echocardiography. The final evidence tables are included
absence of a current authoritative systematic review, in the Online Data Supplement and summarize the
feasibility of defining the benefit and risk in a time frame evidence used by the writing committee to formulate
consistent with the writing of a guideline, relevance to a recommendations. References selected and published
substantial number of patients, and likelihood that the in the present document are representative and not
findings can be translated into actionable recommenda- all-inclusive.
tions. Evidence review committee members may include
methodologists, epidemiologists, clinicians, and bio-
statisticians. Recommendations developed by the writing 1.2. Organization of the Writing Committee
committee on the basis of the systematic review are The writing committee consisted of clinicians, cardiol-
“SR”
marked . ogists, internists, interventionalists, surgeons, radiolo-
gists, anesthesiologists, a nurse practitioner, and a
Guideline-Directed Medical Therapy lay/patient representative. The writing committee
The term guideline-directed medical therapy encom- included representatives from the ACC, AHA, American
passes clinical evaluation, diagnostic testing, and both Association for Thoracic Surgery, American College of
pharmacological and procedural treatments. For these Radiology, Society of Cardiovascular Anesthesiologists,
and all recommended drug treatment regimens, the Society for Cardiovascular Angiography and In-
reader should confirm dosage with product insert mate- terventions, Society of Thoracic Surgeons (STS), and
rial and evaluate for contraindications and interactions. Society for Vascular Surgery (SVS). Appendix 1 of the
Recommendations are limited to drugs, devices, and present document lists writing committee members’
treatments approved for clinical use in the United States. relevant RWI. For the purposes of full transparency,
Joshua A. Beckman, MD, MS, FACC, FAHA the writing committee members’ comprehensive
Chair, AHA/ACC Joint Committee on disclosure information is available in a Supplemental
Clinical Practice Guidelines Appendix.
TABLE 1 Associated Guidelines
Publication Year
Title Organization (Reference)
Guidelines
Thoracic endovascular aortic repair for descending thoracic aortic aneurysms SVS 20211
Valvular heart disease ACC/AHA 20202
Large vessel vasculitis EULAR 20203
Blood cholesterol AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/ 20194

APhA/ASPC/NLA/PCNA
Congenital heart disease AHA/ACC 20195
Abdominal aortic aneurysm SVS 20186
High blood pressure ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/ 20187

PCNA
Lower extremity peripheral artery disease AHA/ACC 20178
Descending thoracic aorta diseases ESVS 20179
Bicuspid aortic valves statement of clarification ACC/AHA 201610
Vascular graft infections, mycotic aneurysms, and endovascular infections AHA 201611
Endovascular repair of traumatic thoracic aortic injury SVS 201112
Thoracic aortic disease ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM 201013
Coronary and other atherosclerotic vascular disease AHA/ACC 200614
Acute type A aortic dissection AATS 202115
Type B Aortic Dissection STS 202216
AACVPR indicates American Association of Cardiovascular and Pulmonary Rehabilitation; AAPA, American Academy of Physician Assistants; AATS, American Association for Thoracic
Surgery; ABC, Association of Black Cardiologists; ACC, American College of Cardiology; ACCF, American College of Cardiology Foundation; ACPM, American College of Preventive
Medicine; ACR, American College of Radiology; ADA, American Diabetes Association; AGS, American Geriatrics Society; AHA, American Heart Association; APhA, American Pharmacists
Association; ASA, American Society of Anesthesiologists; ASH, American Society of Hematology; ASPC, American Society for Preventive Cardiology; ESVS, European Society for Vascular
Surgery; EULAR, European League Against Rheumatism; NLA, National Lipid Association; NMA, National Medical Association; PCNA, Preventive Cardiovascular Nurses Association; SCA,
Society of Cardiovascular Anesthesiologists; SCAI, Society for Cardiovascular Angiography and Interventions; SIR, Society of Interventional Radiology; STS, Society of Thoracic Sur-
geons; SVM, Society for Vascular Medicine; and SVS, Society for Vascular Surgery.
1.3. Document Review and Approval 1.4. Scope of the Guideline

The Joint Committee appointed a peer review com- In developing the “2022 ACC/AHA Guideline for the
mittee to review the document. The peer review Diagnosis and Management of Aortic Disease” (2022 aortic
committee was comprised of individuals nominated by disease guideline), the writing committee reviewed pre-
ACC, AHA, and the collaborating organizations. Re- viously published guidelines. Table 1 contains a list of
viewers’ RWI information was distributed to the these publications deemed pertinent to this writing effort
writing committee and is published in this document and is intended for use as a resource, thus obviating the
(Appendix 2). need to repeat existing guideline recommendations.
This document was approved for publication by the
governing bodies of the ACC and the AHA and was 1.5. Class of Recommendations and Level of Evidence
endorsed by the American Association for Thoracic The Class of Recommendation (COR) indicates the
Surgery, American College of Radiology, Society of strength of recommendation, encompassing the esti-
Cardiovascular Anesthesiologists, Society for Cardiovas- mated magnitude and certainty of benefit in proportion to
cular Angiography and Interventions, Society of Inter- risk. The Level of Evidence (LOE) rates the quality of
ventional Radiology, Society of Thoracic Surgeons, scientific evidence supporting the intervention on the
Society for Vascular Medicine, and Society for Vascular basis of the type, quantity, and consistency of data from
Surgery. clinical trials and other sources (Table 2).1
Applying American College of Cardiology/American Heart Association Class of Recommendation and Level of Evidence
TABLE 2
to Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care* (Updated May 2019)
1.6. Abbreviations
Abbreviation Meaning/Phrase
Abbreviation Meaning/Phrase AVR aortic valve replacement
3D 3-dimensional BAAI blunt traumatic abdominal aortic injury
AAA abdominal aortic aneurysm BAV bicuspid aortic valve
AAS acute aortic syndrome BP blood pressure
ACEI angiotensin-converting enzyme inhibitor BSA body surface area
AHI aortic height index BTAI blunt traumatic aortic injury
AR aortic regurgitation BTTAI blunt traumatic thoracic aortic injury
ARB angiotensin receptor blocker CMR cardiac magnetic resonance
ASCA aberrant subclavian artery CoA coarctation of the aorta
ASCVD atherosclerotic cardiovascular disease CT computed tomography
ASI aortic size index CTA computed tomographic angiography
Continued on the next page Continued on the next page

Abbreviation Meaning/Phrase
junction to the innominate artery; the aortic arch, which
DBP diastolic blood pressure extends from the innominate to the left subclavian artery;
DMARD disease-modifying anti-rheumatic drug
the descending thoracic aorta, which extends from the
left subclavian artery to the diaphragm; and the abdom-
ECG electrocardiogram
inal aorta, which extends from the diaphragm to the level
EVAR endovascular abdominal aortic aneurysm repair
of the aortic bifurcation.
FID focal intimal disruption
The aortic wall is composed of 3 layers (Figure 2): a thin
FDA U.S. Food and Drug Administration
inner intima, a thicker central media, and a thin outer
FDG-PET fluorodeoxyglucose–positron emission tomography
adventitia. The intima consists of a layer of endothelial
FEVAR fenestrated endovascular aortic repair
cells within a matrix of connective tissue. The media
GCA giant cell arteritis consists of smooth muscle cells, elastic fibers, collagen
HRQOL health-related quality of life proteins, and polysaccharides sandwiched in >50 layers
HTAD heritable thoracic aortic disease known as elastic lamellae. The media provides strength
ICU intensive care unit and distensibility to the aorta, features that are critical to
IMH intramural hematoma circulatory function. The adventitia is composed of con-
IRAD International Registry of Acute Aortic Dissection nective tissue, fibroblasts, nerves, and the vasa vasorum,
LDL low-density lipoprotein which perfuse the outer aortic wall and a substantial
LVV large vessel vasculitis
portion of the media.
MR magnetic resonance
MRA magnetic resonance angiography

2.2. Aortic Landing Zones
MRI magnetic resonance imaging
In addition to the standard anatomic descriptors of the
nsHTAD nonsyndromic heritable thoracic aortic disease
aortic anatomy, there is a more technical classification of
PAD peripheral artery disease
aortic anatomy that is used to plan, guide, and report
PAU penetrating atherosclerotic ulcer
aortic interventions, especially endovascular stent-
PET positron emission tomography grafting. Because the clinical success of thoracic aortic
rAAA ruptured abdominal aortic aneurysm endovascular procedures is influenced by the proximal
RCT randomized controlled trial sealing zone, in this system the thoracic and abdominal
REBOA resuscitative endovascular balloon occlusion of the aorta aorta are divided into 11 landing zones, as detailed in
rEVAR endovascular repair for rAAA Figure 3.
SMA superior mesenteric artery Note that Roselli et al 2 have proposed a modification of
SBP systolic blood pressure zone 0, dividing it into 3 subsegments, in which 0A ex-
SCI spinal cord injury tends from the annulus to the distal margin of the highest
coronary, 0B extends above the coronary to the distal
TAA thoracic aortic aneurysm
margin of the right pulmonary artery, and 0C extends
TAAA thoracoabdominal aortic aneurysm
from the right pulmonary artery to the distal end of the
TAAD thoracic aortic aneurysm and dissection
origin of the innominate artery.
TAD thoracic aortic disease
TAR total arch replacement 2.3. Definitions of Dilation and Aneurysm of the Aortic Root
TEE transesophageal echocardiography and Ascending Thoracic Aorta
TEVAR thoracic endovascular aortic repair The conventional definition of an arterial aneurysm is any
TTE transthoracic echocardiography artery that is dilated to at least 1.5 times its expected
VSRR valve-sparing root replacement normal diameter. 3 This definition applies well to the
abdominal and descending thoracic aorta. However, it has
long been recognized that this definition fails when it
2. NORMAL ANATOMY, ABNORMAL ANATOMY, comes to defining aneurysms of the aortic root and
AND DEFINITIONS ascending thoracic aorta. For example, a man in his 40s
would be expected to have an average aortic root diam-
2.1. Normal Aortic Anatomy eter of 3.5 cm; applying the standard definition of $1.5
The aorta is the largest artery in the body and can be times reference diameter, his aortic root would have to
divided into 5 main anatomic segments (Figure 1): the root reach 5.25 cm before it would be considered an aneurysm,
or sinus segment, which extends from the aortic valve whereas most experts would consider his aorta to be an
annulus to the sinotubular junction; the ascending aneurysm well below that diameter. Indeed, if this patient
thoracic aorta, which extends from the sinotubular had Marfan syndrome or a familial thoracic aortic
F I G U R E 1 The Anatomy of the Aorta and Its Main Branches

F I G U R E 2 A Simplified Diagram Depicting the Key Histologic Components of the Aortic Wall
The medial layer in human aortas contains >50 alternating layers of elastin and smooth muscle cells (whereas only 5 are shown in this simplified illustration).
Adapted (cropped) from "Illustration of tunics of the arteries vs veins" by Malgosia Wilk-Blaszczak, used under CC-BY 4.0. "Illustration of tunics of the
arteries vs veins" is adapted (cropped) from figure 20.3 in BC OpenStax Anatomy and Physiology used under CC-BY 4.0.
aneurysm, aortic repair would be recommended at a population at risk at each aortic diameter (denominator).
diameter of #5.0 cm, a size that would not even be large They found that, relative to a control aortic diameter
enough to be termed an “aneurysm.” of #3.4 cm, a diameter of 4.0 cm to 4.4 cm conferred an
The most important consideration in deciding the 89-fold increased risk of dissection, and a diameter
diameter thresholds at which to call the root and of $4.5 cm conferred a 6,000-fold increased risk
ascending aorta dilated or aneurysmal is based on the (Figure 5), albeit these are only relative risk estimates and
natural history of such abnormal aortas. Borger et al4 do not inform absolute risk. It follows that the increase in
studied 201 patients with bicuspid aortic valve (BAV) risk at 4.0 cm to 4.4 cm justifies defining an aorta of this
undergoing aortic valve replacement (AVR) (those un- size “dilated,” and the abrupt increase in risk at a diam-
dergoing concomitant replacement of the ascending aorta eter of $4.5 cm justifies defining an aorta of this size as an
were excluded) and followed them for 10 to 15 years; they “aneurysm.” Using this approach, of the subjects in the
found that those with baseline aortic diameters of 4.5 cm MESA database, only 2.6% would be considered to have a
to 4.9 cm had a significantly increased risk of aneurysm, dilated aorta and only 0.2% to have an aneurysm.
dissection, or sudden death (P<0.001) compared with This definition of a dilated ascending aorta being $4.0
those with diameters <4.5 cm (Figure 4). cm is consistent with what was proposed in the 2014
To evaluate the risk of type A aortic dissection at European Society of Cardiology guidelines on the diag-
various diameters below the traditional 5.5 cm threshold nosis and treatment of aortic diseases, in which aortic
for prophylactic aortic repair, Paruchuri et al5 plotted a “dilation” was similarly defined as an aorta diameter of
distribution curve of ascending aortic size in a community >4.0 cm.6
sample from the MESA (Multi-Ethnic Study of Athero- Finally, in the clinical setting, the term “dilation” is
sclerosis) database. They then analyzed the number of preferred to “ectasia” to describe mild aortic enlarge-
dissections (numerator) at each aortic diameter and the ment. Historically, there has been a lack of uniformity in
“ectasia” is typically used to describe diffuse (rather than

F I G U R E 3 Classification of Aortic Anatomic Segments by
11 Landing Zones
focal) coronary artery dilation,9 which may lead to some
clinical uncertainty when “ectasia” is applied to the aorta.
2.3.1. Normalizing Aortic Root and Ascending Aortic Diameters

for Body Size
As with the aortic diameter thresholds for surgery pre-
sented in this guideline, it recognized that the 4.0 cm and
4.5 cm diameter thresholds discussed previously are
intended for those whose height, body surface area (BSA),
or both is within 1 to 2 standard deviations of the mean.
For male and female patients who are significantly shorter
or taller than average, these diameters need to be
adjusted downward or upward, accordingly. Several
methods to normalize aortic diameter are currently used
in clinical practice and clinical research.
The Z-Score
The z-score is routinely used to assess aortic dilation in
the pediatric population, as changes in a child’s age and
body size make it especially challenging to define normal
aortic size and to distinguish normal from pathologic
aortic growth. Nomograms have been established corre-
lating BSA and aortic root diameter to generate the z-
score. One limitation of the reliance on BSA is that there
are multiple formulae to calculate BSA that yield different
results for the same patient. A second limitation is that
multiple z-score calculators exist, each performing
Zone 0 (involves the ascending to distal end of the origin of the
innominate artery); Zone 1 (involves the origin of the left common
differently.10 Finally, most of the literature on the natural
carotid; between the innominate and the left carotid); Zone 2 (in- history of acute aortic syndromes (AAS) is based on aortic
volves the origin of the left subclavian; between the left carotid and diameters, whereas reports of outcome based on z-scores
the left subclavian); Zone 3 (involves the proximal descending are limited, so the z-score is not typically used to report
thoracic aorta down to the T4 vertebral body; the first 2 cm distal
the degree of aortic dilation in adults.
to the left subclavian); Zone 4 (the end of zone 3 to the mid-
descending aorta – T6); Zone 5 (the mid-descending aorta to the
The Aortic Size Index and Aortic Height Index
celiac); Zone 6 (involves the origin of the celiac; the celiac to the Most often, in the clinical care of adult patients, aortic
superior mesenteric); Zone 7 (involves the origin of the superior diameters are normalized using a ratio of aortic diameter
mesenteric artery; the superior mesenteric to the renals); Zone 8 to BSA or aortic diameter to the patient’s height. In 2006,
(involves the origin of the renal arteries; the renal to the infrarenal
Davies et al11 showed that aortic size index (ASI), which is
abdominal aorta); Zone 9 (the infrarenal abdominal aorta to the
level of aortic bifurcation ); Zone 10 (the common iliac); Zone 11
defined as aortic diameter (cm)/BSA(m 2), is a better pre-
(involves the origin of the external iliac arteries). From Czerny et al.1 dictor of adverse aortic events than diameter alone, and
Copyright 2019, with permission from Elsevier, Inc., Now Medical that a simple nomogram could be used to stratify those
Studios, and Oxford University Press on behalf of the European with aortic aneurysms into low-, medium-, and high-risk
Association for Cardio-Thoracic Surgery.
groups. However, it is unclear whether the weight of an
adult has a significant impact on the expected normal
aortic diameter, and one would not expect a patient’s
aorta to grow or shrink with significant fluctuations in
the use of “ectasia” in image interpretation. Many radi- weight. Zafar et al12 therefore examined whether aortic
ologists use “ectatic” rather than “dilated” to describe a height index (AHI), which is defined as aortic diameter
mildly enlarged aorta, whereas others use “ectatic” to (cm)/patient height (m), might perform better than the
describe an abnormal aortic shape, such as a “tortuous” ASI, and they reported that the AHI performed at least as
aorta.7 Even more problematic is the fact that some im- well as the ASI 12 and had the advantage of being simpler
aging groups use the term “ectasia” to describe larger to calculate.
aortas, such as those 4.5 cm to 5.4 cm in diameter,8 which The Cross-Sectional Area to Height Ratio
overlaps with what most experts would consider to be an Another approach to normalizing aortic size to
aneurysm. Lastly, in imaging of the coronary arteries, height was proposed by Svensson et al in 2002 13 in
F I G U R E 4 Freedom From Ascending Aortic Complications for Patients With Bicuspid Aortic Valve Disease
Patients with moderate dilation of the ascending aorta (4.5 cm–4.9 cm) had a significantly increased risk of future aortic complications (aneurysm, dissection,
or sudden death). Reprinted from Borger et al.4 Copyright 2004, with permission from Elsevier, Inc. and the American Association for Thoracic Surgery.
which they calculated a ratio of the cross-sectional adverse events; notably, in more contemporary re-
area of the aorta (cm) to the patient’s height (m). ports, this group has shown the simpler cross-sectional
The initial studies used a cross-sectional area to area to height ratio of $10 cm 2/m (rather than
2 2
height ratio of >10 cm /m as a threshold for inter- >10 cm /m) as the threshold predictive of increased
vention because of a significant increase in risk of risk. 14,15
F I G U R E 5 Relative Risk of Aortic Dissection by Size Range
The relative risk of aortic dissection begins to increase appreciably at a diameter of 4.0 cm to 4.4 cm and then increases dramatically at a diameter
of $4.5 cm. Reprinted from Paruchuri et al.5 Copyright 2005, with permission from Karger Publishers, Basel Switzerland.
F I G U R E 6 Acute Aortic Syndromes
In aortic dissection, a tear in the aortic intima allows blood to penetrate the aortic media, pushing the dissection flap into the middle of the aorta, separating
the true from the false lumen. In intramural hematoma, blood leaks into the aortic media at low pressure, forming a thrombus that pushes the outer wall of
the aorta outward, leaving a relatively normal appearing aortic lumen. A penetrating atherosclerotic ulcer allows blood to enter the aortic media, but
atherosclerotic scarring of the aorta typically confines the blood collection, often resulting in a localized dissection or pseudoaneurysm. Adapted with
permission from Springer Nature Customer Service Centre GmbH: Springer Nature, Clough et al1 Copyright 2015.
2.4. Definitions and Classification of Acute Aortic Syndrome back through the intima into the true lumen, creating a
(AAS) reentry tear. If the blood in the false lumen instead tears
AAS are life-threatening conditions in which there is a through the outer media and adventitia, aortic rupture
breach in the integrity of the aortic wall. The most com- will result. The incidence of aortic dissection is estimated
mon AAS are aortic dissection, intramural hematoma to be 5 to 30 cases per million people per year, with men
(IMH), and penetrating atherosclerotic ulcer (PAU), all of more commonly affected. Most dissections occur in those
which can lead to rupture (Figure 6). between the ages of 50 to 70 years, although patients with
Marfan syndrome, BAV, Loeys-Dietz syndrome, and
2.4.1. Aortic Dissection vascular Ehlers-Danlos syndrome, present at younger
Aortic dissection is the most common of the AAS. Aortic ages.
dissection occurs when there is an intimal tear that allows
the blood to pass through the tear and into the aortic 2.4.1.1. Definition
media, splitting the intima in 2 longitudinally, creating a Aortic dissection has traditionally been defined as “acute”
dissection flap that divides the true lumen from a newly during the first 2 weeks after symptom onset and
formed false lumen (Figure 6). The dissection flap can “chronic” when beyond the second week. Investigators
propagate in an antegrade or retrograde fashion and lead from the International Registry of Acute Aortic Dissection
to a number of life-threatening complications, including (IRAD) proposed that aortic dissection be divided into 4
acute aortic regurgitation (AR), myocardial ischemia, temporal types: hyperacute (<24 h), acute (2–7 d), sub-
cardiac tamponade, acute stroke, or malperfusion syn- acute (8–30 d), and chronic (>30 d). 2 The most contem-
dromes. The blood surging in the false lumen may rupture porary temporal classification system, proposed by the
Classification of Aortic Dissection Chronicity

with an early mortality of 1% to 2% per hour after
TABLE 3
Based on the 2020 SVS/STS Reporting Standards symptom onset.3 The mortality rate is increased
among patients who present with or develop compli-
Chronicity Time From Onset of Symptoms
cations of cardiac tamponade (with or without
Hyperacute <24 h
cardiogenic shock), acute myocardial ischemia or
Acute 1–14 d
infarction, stroke, or organ malperfusion. 3 Patients
Subacute 15–90 d
with uncomplicated acute type B aortic dissection
Chronic >90 d
have a 30-day mortality rate of 10%. However, when
Adapted with permission from Springer Nature Customer Service Centre GmbH: patients with acute type B aortic dissection develop
Springer Nature, Clough RE, et al.1 Copyright 2015.
complications, such as malperfusion or rupture, the
STS indicates Society of Thoracic Surgeons; and SVS, Society for Vascular Surgery.
mortality rate increases to 20% by day 2 and to 25%
by day 30. 3
SVS and STS, similarly divides aortic dissection into 4
temporal types, as shown in Table 3, to improve prog-
nostication and guide decision making about the timing 2.4.1.2. Classification
and types of potential intervention. There are 2 commonly used anatomic classification sys-
Acute aortic dissection of the ascending aorta is tems for aortic dissection (Figure 7): the DeBakey system
highly lethal in symptomatic patients left untreated, and the Stanford system.
F I G U R E 7 Classification of Acute Aortic Dissection
The DeBakey and Stanford classification systems are used most commonly. The DeBakey system offers greater anatomic detail, whereas the Stanford system
is simpler, essentially distinguishing those dissections that involve the ascending thoracic aorta from those that do not.
F I G U R E 8 Anatomic Reporting of Aortic Dissection Based on the 2020 SVS/STS Reporting Standards
STS indicates Society of Thoracic Surgeons; and SVS, Society for Vascular Surgery. Reprinted from Lombardi et al.5 Copyright 2020, with permission from
Elsevier, Inc., the Society of Thoracic Surgeons, and the Society for Vascular Surgery.
The DeBakey system categorizes dissections into types published an expert consensus document4 for the treat-
I, II, and III, based on the origin of the intimal tear and the ment of thoracic arch pathologies, in which they added a
extent of the dissection: third category called “non-A-non-B dissection,” to be
used for patients whose proximal dissection flap begins in
n Type I: Dissection tear originates in the ascending
the aortic arch.
aorta and propagates distally to include the aortic arch
Most recently, in 2020, the SVS and the STS proposed
and typically the descending aorta
an entirely new classification scheme that defines the
n Type II: Dissection tear is confined only to the
aortic dissection anatomy in more granular detail 5: Dis-
ascending aorta
sections are defined anatomically according to the loca-
n Type III: Dissection tear originates in the descending
tion of intimal tears and the proximal and distal extent of
thoracic aorta and propagates most often distally
the dissection process (Figure 8).
n Type IIIa: Dissection tear is confined only to the
A D indicates type A is used for any dissection with
descending thoracic aorta
an entry tear in zone 0 and extends distally the zone
n Type IIIb: Dissection tear originates in the descending
denoted by the subscript D (eg, A 9); BPD, type B is used
thoracic aorta and extends below the diaphragm
for any dissection with an entry tear in zone 1 or
The Stanford classification system divides dissections beyond; the proximal and distal extents of the dissec-
into 2 categories according to whether the ascending aorta tion are denoted by subscripts P and D, respectively
is involved or not, regardless of the site of origin: (eg, B 39 ). I D, when a dissection begins in zone 0 but the
location of the entry tear has not been identified, it will
n Type A: All dissections involving the ascending aorta,
be considered “Indeterminate”; it will be designated
irrespective of the site of the intimal tear
with an I and its distal extent denoted by the subscript
n Type B: All dissections that do not involve the
D (eg, I 9).
ascending aorta (including dissections that involve the
aortic arch but spare the ascending aorta) 2.4.1.3. Malperfusion
In 2019, the European Association for Cardio-Thoracic Malperfusion syndrome occurs when there is end-organ
Surgery and the European Society for Vascular Surgery ischemia related to inadequate perfusion of the aortic
branch vessels. The relationship of the true and false lu-

F I G U R E 9 Mechanisms of Dynamic and Static Obstruction in Aortic
mens in an aortic dissection has a critical role in main- Dissection
taining stable perfusion of end-organs. Initially, the true
lumen collapses because of the loss of transmural pres-
sure across the dissection flap and the subsequent elastic
recoil of the medial smooth muscle. Simultaneously, the
false lumen expands immediately because of reduced
elastic recoil, depth of the dissection plane within the
media, and percentage of the wall circumference
involved. Any of the aortic branches are at risk for mal-
perfusion as the false lumen expands and compresses the
true lumen and can occur in multiple vascular beds
simultaneously as the dissection propagates distally. Dy-
namic obstruction occurs when the septum of the
dissected intima prolapses across into the ostia of a
branch, usually during systole, thereby not allowing
adequate flow to perfuse the vessel (Figure 9). The ostia
itself remains anatomically undamaged. When the
dissection tear extends into the vessel proper and creates
a stenosis or thrombosis in the artery, static obstruction
occurs (Figure 9).
2.4.2. Intramural Hematoma

IMH describes the presence of blood within the medial
layer of the aortic wall in the absence of an overt intimal
tear or patent false lumen. The blood may arise from
either rupture of the vasa vasorum causing bleeding
within the media7 or small intimal tears that are not
visualized on standard imaging exams. 8 IMH is diagnosed
by computed tomographic angiography (CTA), magnetic
resonance imaging (MRI), and echocardiography by the
(A) Static obstruction occurs when the dissection flap extends from the
presence of a circular or crescent-shaped thickening of
aortic lumen into the ostium of the affected branch vessel, leading to
the aortic wall of >5 mm in the absence of detectable localized thrombosis of the branch false lumen that narrows or col-
blood flow 9 (Figure 6). Of patients presenting with sus- ludes the branch true lumen and, consequently, impairs distal branch
pected AAS, studies suggest that 5% to 25% have IMH, a perfusion. (B) Dynamic obstruction occurs when the false lumen be-
proportion that approaches 30% to 40% in the Asian comes persistently pressurized and compresses the true lumen, in turn
8-11 pushing the dissection flap up against the ostium of the affected
literature.
branch vessel, significantly reducing or occluding its flow. (C) Some-
The natural history of IMH is variable. Fewer than 10% times, a branch vessel can suffer from both static and dynamic
of IMH cases resolve spontaneously, whereas 16% to 47% obstruction at the same time. Adapted with permission from Grewal
progress to aortic dissection if the intimal layer ruptures et al.6 Copyright 2021, Elsevier, Inc.
7,12
and creates an entry tear.
2.4.3. Penetrating Atherosclerotic Ulcer

A PAU begins with an ulceration of an atherosclerotic
plaque, which leads to a focal disruption in the aortic PAUs can vary in size, and often multiple PAUs are pre-
intima that allows blood to penetrate into the medial layer sent.10 The true incidence is unknown but is estimated to
and is often associated with an IMH of variable size.10 account for 2% to 7% of all cases of AAS. 10 Typically, pa-
PAUs most often appear in the middle or distal descend- tients with PAU are older (>70 years of age) than those
ing thoracic aorta, less frequently in the aortic arch and with classic aortic dissection and present more often with
abdominal aorta, and rarely in the ascending aorta.8,10 extensive and diffuse atherosclerotic disease involving
F I G U R E 1 0 Classification of Thoracoabdominal Aortic Aneurysms
The classification of thoracoabdominal aortic aneurysms according to extent of aortic involvement as originally proposed by Crawford is as follows3: Extent I,
below the left subclavian to above the celiac axis or opposite the superior mesenteric and above the renal arteries; Extent II, below the left subclavian and
including the infrarenal abdominal aorta to the level of the aortic bifurcation; Extent III, below T6 intercostal space, tapering to just above the infrarenal
abdominal aorta to the iliac bifurcation; and Extent IV, below T12, tapering to above the iliac bifurcation. Safi et al1 proposed expanding the classification with
the addition of Extent V, below T6, tapering to just above the renal arteries.
both the aorta and coronary arteries.10 Additional com- 2.5. Classification of Thoracoabdominal Aortic Aneurysm
mon comorbidities include hypertension, tobacco use, (TAAA)
chronic obstructive pulmonary disease, and renal insuf- When descending thoracic aortic aneurysms (TAA)
ficiency. PAU can occur in younger patients but often in extend into the abdominal aorta, they are referred to as
the setting of a connective tissue disorder, and men are thoracoabdominal aortic aneurysms (TAAA). The Craw-
more commonly affected than women. 8 ford classification of TAAA, later modified by Safi et al1
The natural history of PAU is not well defined, as (Figure 10), not only describes the extent of
they can remain stable, enlarge, or progress to either an aneurysm but also may predict the morbidity and
IMH, dissection, pseudoaneurysm, or aortic rupture.8 mortality associated with aneurysm repair. 2
The risk of rupture has been reported to be up to
40%.13 The optimal management strategy must be
individualized, considering the clinical presentation, 2.6. Classification of Endoleaks
the imaging features of the PAU, and the patient’s Endovascular stent-grafting is widely used in the repair of
comorbidities. aortic aneurysms. One of the limitations of endografting
F I G U R E 1 1 Classification of Endoleak Types
Endoleaks are classified by 5 types: Type Ia, proximal attachment site endoleak; Type Ib, distal attachment site endoleak; Type II, backfilling of the aneurysm
sac through branch vessels of the aorta; Type III, graft defect or component misalignment; Type IV, leakage through the graft wall attributable to endograft
porosity; and Type V, caused by “endotension,” possibly resulting from aortic pressure transmitted through the graft/thrombus to the aneurysm sac. Adapted
from Rokosh et al.2 Copyright 2021, with permission from Elsevier, Inc., and the Society for Vascular Surgery.
is the occurrence of endoleaks, either early or late aneurysm sac, preventing its complete thrombosis.
following the procedure. There are 5 types of endoleaks, Consequently, patients with endografts require lifelong
as detailed in Figure 11. An endoleak results in the surveillance imaging to monitor for the appearance of
persistence of blood flow outside the graft and within the endoleaks. 1
3. IMAGING AND MEASUREMENTS
3.1. Aortic Imaging Techniques to Determine Presence and

Progression of Aortic Disease
Recommendations for Aortic Imaging Techniques to Determine Presence and Progression of Aortic Disease
Referenced studies that support the recommendations are summarized in the Online Data Supplement.
COR LOE RECOMMENDATIONS
1. In patients with known or suspected aortic disease, aortic diameters should be measured at reproducible
1 B-NR anatomic landmarks perpendicular to axis of blood flow, and these measurement methods should be
reported in a clear and consistent manner. In cases of asymmetric or oval contour, the longest diameter
and its perpendicular diameter should be reported.3,4
2. In patients with known or suspected aortic disease, episodic and cumulative ionizing radiation doses
1 C-LD should be kept as low as feasible while maintaining diagnostic image quality. 5-7
3. In patients with known or suspected aortic disease, when performing CT or MR imaging, it is recom-
1 C-EO mended that the root and ascending aortic diameters be measured from inner-edge to inner-edge, using
an electrocardiographic-synchronized technique. If there are aortic wall abnormalities, such as athero-
sclerosis or discrete wall thickening (more common in the distal aorta), the outer-edge to outer-edge
diameter should be reported (Table 4).
4. In patients with known or suspected aortic disease, the aortic root diameter should be recorded as
1 C-EO maximum sinus to sinus measurement. In the setting of known asymmetry, multiple measurements
should be reported, and both short- and long-axis images of the root should be obtained to avoid un-
derestimation of the diameter.
5. In patients with known or suspected aortic disease, it is reasonable that a dilated root or ascending aorta
2a C-LD be indexed to patient height or BSA in the report, to aid in clinical risk assessment. 8-11
6. In patients with known or suspected aortic disease, when performing echocardiography, it is reasonable
2a C-EO to measure the aorta from leading-edge to leading-edge, perpendicular to the axis of blood flow.
Using inner-edge to inner-edge measurements may also be considered, particularly on short-axis

2b C-EO imaging.
Synopsis combined with rapid acquisition of intuitive, high-

Optimized depiction of aortic anatomy and pathology resolution 3-dimensional (3D) imaging data sets, has led
requires dedicated aortic imaging protocols. Computed to the wide adoption of this modality for the assessment
tomography (CT), magnetic resonance imaging (MRI), of suspected aortic pathology and for periprocedural
transthoracic echocardiography (TTE) and trans- vascular evaluation, in most cases supplanting diagnostic
esophageal echocardiography (TEE), and abdominal catheter angiography.12
aortic ultrasound all have important roles in these eval-
Recommendation-Specific Supportive Text
uations (Table 5). Selection of an imaging modality may be
based on patient-specific factors, including hemodynamic 1. Measurements should be obtained perpendicular to the
stability, contrast allergy, renal function, and patient long axis of the aorta at specified segmental locations
tolerance (eg, given relatively longer examination times (Figure 12), with measurements also taken at the loca-
and the confined space associated with MRI, occasionally tions of any abnormalities. If a 3D data set has been
requiring sedation). The institutional availability of an acquired, dedicated multiplanar reformats orthogonal
imaging modality or an expert imaging physician may also to aortic flow axis should be created at each level of
direct modality selection. The ubiquity of CT scanners, measurement. This approach provides structured,
TABLE 4 Essential Elements of CT and MRI Aortic Imaging Reports
1. Maximum aortic diameter at each level of dilation, perpendicular to the axis of blood flow. In cases of asymmetric or oval contour, the longest diameter and
its perpendicular diameter should be reported. Standard measurement levels may be included, even when normal.
2. Wall changes suggestive of atherosclerosis, diffuse thickening (eg, aortitis), or mural thrombus.
3. Evidence of luminal stenosis/occlusion, including location, severity, and length.
4. Findings suggestive of acute aortic syndrome (eg, communicating dissection, intramural hematoma, penetrating atherosclerotic ulcer, focal intimal tear),
including proximal/distal extension (Figure 7), suspected entry tear site (if visible), and complications (eg, active contrast extravasation, rupture, contained
rupture, rupture including periaortic hemorrhage, pericardial and pleural fluid, mediastinal stranding).
5. Extension of aortic disease process (acute or chronic) into branch vessels, findings suggestive of end-organ injury, and suspected malperfusion.
6. Direct comparison with previous examinations should be detailed to identify pertinent changes.
7. Presence and extent of repair (eg, interposition graft, endovascular stent graft), as well as any evidence of complication.
8. Impression regarding disease classification (eg, acute aortic syndrome, aneurysm/pseudoaneurysm, luminal stenosis, atherosclerotic aortic disease).
9. Relevant details regarding method of image acquisition (eg, use of electrocardiographic-gating and phase of acquisition) and measurement (eg, axial versus
double oblique, inner-edge versus outer-edge) should be included.
CT indicates computed tomography; and MRI, magnetic resonance imaging.
repeatable measurement reporting on serial imaging confidence in the determination of aortic root margins
and avoids oblique imaging that may overestimate the on MRI and lower interobserver and intraobserver
aortic diameter at levels of greater curvature and variability.15 Measurement of graft material (eg, inter-
tortuosity. 3,4 posed surgical or endostent) may likewise include an
2. The cancer risk associated with CT scans remains a inner-edge to inner-edge measurement for determi-
controversial issue; however, the risk is generally nation of the functional lumen and potential use in
agreed to be greatest early in life and substantially extension treatment planning. The use of
attenuated later in life.5,6 Consideration of the indica- electrocardiographic-gated images decreases motion
tion for aortic imaging, optimization of the tube set- artifact and improves edge depiction in aortic root
tings for CT protocols, and use of alternative imaging, with diminished measurement variability.16 If
modalities such as MRI are all valid approaches to there are aortic wall changes (eg, atherosclerosis,
mitigate patient radiation exposure.7 mural thrombus), as is more commonly noted in the
3. On CT and MRI, the root diameter can be measured arch and distal aorta, or discrete wall thickening (eg,
from the commissure to the opposite sinus, or from aortitis or IMH), the outer margins of the abnormal
sinus to sinus, which results in larger dimensions segments are measured.
(Figure 12).13 Measuring from sinus to sinus and from 4. The shape of the aortic root can be asymmetric, and the
inner-edge to inner-edge on CT and MRI has shown difference between the minimum (short-axis) and
good correlation with TTE for measurements of the maximum (long-axis) root diameters can be significant,
root and ascending segments,14 as well as improved particularly in those with bicuspid valves. 17 To avoid
TABLE 5 Diagnostic Performance of Aortic Imaging Modalities
Parameter CT MRI TTE TEE US
Availability þþþ þþ þþþ þþ þþþ
Portability - - þþþ þþþ þþþ
Speed of acquisition þþþ þ þþ þþ þþ
Spatial resolution þþþ þþ þþ þþþ þþ
Temporal resolution þ þþ þþþ þþþ þþþ
Three-dimensional data set þþþ þþ þ þ þ
Arch branch vessel evaluation þþþ þþþ þþ þ NA
Evaluation of valve and ventricular function þ þþ þþþ þþþ NA
CT indicates computed tomography; MRI, magnetic resonance imaging; NA, not applicable; TEE, transesophageal echocardiography; TTE, transthoracic echocardiography; US,
abdominal aortic ultrasound; þþþ excellent results; þþ good results; þ fair results; and -, not available.
F I G U R E 1 2 Aortic Imaging Techniques to Determine the Presence and Progression of Aortic Disease
(A) Schematic shows the leading-edge to leading-edge measurement technique used in echocardiography, from left to right: measurement of the aortic root
(sinuses of Valsalva), sinotubular junction, and proximal tubular ascending aorta. (B) Inner-wall to inner-wall measurements of the aortic root used in MRI
and CT. In addition, a consistent approach to measuring all 3 sinuses with MRI and CT is necessary. The sinus-to-commissure and sinus-to-sinus mea-
surements can both be used, but consistency is necessary for interval surveillance. (C) Standard measurement locations for MRI and CT with the inner-wall to
inner-wall technique. Adapted from Borger et al.21 Copyright 2018, with permission from Elsevier, Inc. CT indicates computed tomography; and MRI,
magnetic resonance imaging. *Leading-edge to leading-edge. †Inner-wall to inner-wall.
underestimation, multiple measurements should be 6. There is a wealth of historical data regarding using TTE
reported, with either each of the sinus-to-sinus di- to measure the aortic root (at end-diastole) from the
ameters or both short- and long-axis diameters, to leading-edge of the anterior wall to the leading-edge of
avoid underestimation of the true root size. the posterior wall, identifying the largest diameter.18,19
5. The cross-sectional aortic area to patient height ratio These data led to the determination of normal limits
has been shown to be associated with risk of aortic adjusted for age, sex, and body size20 and provided
dissection and death in patients with tricuspid or insight regarding the prevalence and prognostic
bicuspid valves9,10 (see Section 2.3.1, “Normalizing importance of aortic dilation. Additionally, measuring
Aortic Root and Ascending Aortic Diameters for Body from leading-edge to leading-edge on TTE has shown
Size”), and both ASI and AHI have been shown to good correlation with inner-edge to inner-edge mea-
predict risk of adverse events (rupture, dissection, or surements obtained on CT and MRI. 14 The method of
11
death). inner-edge to inner-edge measurement on TTE images
F I G U R E 1 3 Reformatted CT Image Orthogonal to the Aortic Root at the Level of the Sinuses of Valsalva
The root diameter can be measured from sinus-to-sinus (S-S) or sinus-to-commissure (S-C). The aortic root area (A) can also be measured. CT indicates
computed tomography; and ROI, region-of-interest.
may also be considered, with some experienced in- Although aortic dilation as measured by diameter is a
vestigators showing excellent measurement well-known risk factor for the occurrence of aortic
agreement. 15 dissection and rupture,8 most dissections occur in aortas
3.2. Conventions of Measurements with diameters that do not meet the threshold for pre-
Reproducible and accurate measurements of the aorta are ventive surgery.9 This has led investigators to search for
critical for characterizing aortic disease and guiding better metrics for risk stratification and treatment guid-
treatment decisions. Measurements should be obtained ance. For instance, research has shown that ascending
perpendicular to the long axis of the aorta at specified aortic area indexed to height is associated with aortic
segmental locations (Figure 13),1 with measurements also dissection and adverse outcomes in patients with
taken at the location of any abnormality. Unfortunately, tricuspid or bicuspid valves.10,11 Male sex, age, height,
there is no widely accepted standard for aortic diameter weight, and the presence of traditional cardiovascular risk
measurements (eg, inner-edge to inner-edge, outer-edge factors have also been found to correlate with increased
to outer-edge) across imaging modalities. There is a aortic size in large population-based studies. 12 Aortic
wealth of historical data regarding using TTE to measure length is known to increase over time; spurred by this
the aortic root (at end-diastole) from the leading-edge of fact, and by the observation that intimal entry tears run in
the anterior wall to the leading-edge of the posterior wall, a transverse direction, researchers have found that
thus identifying the largest diameter. 2,3 These data excessive elongation of the ascending aorta may be pre-
allowed for the creation of normal limits adjusted for age, dictive of dissection and thus represents a potentially
sex, and body size 4 and provided insight regarding the relevant measurement.13
prevalence and prognostic importance of aortic dilation. Measurements of the arch and further distal segments
On CT and MRI, the root diameter can be measured should also be performed perpendicular to the aortic axis,
from the commissure to the opposite sinus, or from sinus with care taken to avoid oblique imaging that may over-
to sinus, which results in larger dimensions (Figure 13).5 estimate the aortic diameter at levels of greater curvature
Measuring from sinus-to-sinus and from inner-edge to and tortuosity. In the setting of wall changes (eg, discrete
inner-edge on CT and MRI has shown good correlation thickening from atherosclerosis, aortitis, IMH, or other
with TTE for measurements of the root and ascending processes), the abnormal wall should be measured from
segments,6 as well as improved confidence in the delin- outer-edge to outer-edge. To assess abdominal aortic di-
eation of aortic root margins on MRI and lower interob- mensions, ultrasonographic images may be obtained in a
server and interobserver variability. 7 dedicated examination or as part of a surface
echocardiographic examination. Several studies have of inflammation1 and AAS, and offers physiologic assess-
shown that the volume of an AAA may progress despite a ment of ventricular and valve function plus flow quanti-
stable diameter. 14,15 fication. MRI uses no ionizing radiation and can often be
performed without intravenous contrast. MRI is therefore
3.2.1. Computed Tomography often a primary option for assessing congenital aortic
CT can image the entire aorta and its branches with high abnormalities and is well-suited for serial imaging in
spatial resolution and fast acquisition. The use of younger patients. The use of electrocardiographic-gated
electrocardiographic-gated technique decreases motion imaging decreases motion artifact of the aortic root 2 and
1
artifact of the root and ascending aorta, significantly of 3D datasets, critical for achieving precise, repeatable
increasing the precision of measurements and diagnostic measurements. 3 Limitations of MRI include spatial reso-
confidence. When necessary, CT can be performed lution that, although good, is typically inferior to that of
without the use of iodinated contrast, and such non- CT, as well as the appearance of artifacts in patients with
contrast imaging can still accurately provide diameter indwelling metallic material or devices. Additionally, MRI
assessment of aortic aneurysms that can suffice for sur- is not as widely available as CT for aortic imaging, has a
veillance of patients who cannot tolerate or cooperate longer acquisition time, and the ability to monitor and
with MRI, although aortic wall delineation may be chal- treat unstable patients in the scanner is limited. This
lenging in some instances (eg, at the aortic root level). The modality is therefore less commonly used in patients with
use of iodinated intravenous contrast allows for delinea- suspected acute aortic pathology, 4 especially when pa-
tion between aortic lumen and wall and generally im- tients are unstable. Various MRI sequences are available
proves assessment of wall changes. In some instances, the for aortic depiction, including magnetic resonance angi-
potential concern of patient contrast allergy or renal ography (MRA), which involves volumetric acquisition of
toxicity may be a consideration. However, according to aortic anatomy, with slice thickness allowing for recon-
recent consensus statements from the American College struction of images in multiple planes. Intravenous
of Radiology and the National Kidney Foundation,2 the gadolinium-based contrast media are often used in MRA,
risk of acute kidney injury developing in patients with although there is a very small risk of inducing nephro-
impaired renal function after exposure to intravenous genic systemic fibrosis in patients with underlying kidney
iodinated contrast media has likely been overestimated disease, a risk that is particularly low with group II gad-
given the difficulty distinguishing coincident from olinium-based contrast agents. 5,6 Additional sequences
contrast-induced nephropathy. are often used for aortic anatomic depiction that do not
CT has a very high sensitivity and specificity for acute require intravenous contrast media, such as cine gradient
aortic syndromes (AAS, aortic dissection, IMH, PAU) 3 and echo bright blood and spin echo dark blood sequences.
traumatic aortic injuries. Moreover, CT can identify For consistency in this document, we use MRI to refer to
concomitant coronary involvement, 4 branch vessel the modality of magnetic resonance imaging defined
involvement, and hemopericardium, and may aid in broadly, which potentially includes many sequences that
identification of dissection entry tears. In patients whose are often combined in complementary manner within an
CT is negative for AAS, the images may provide insight imaging protocol.
regarding other causes of the presenting chest pain. 5
When imaging patients with a suspected AAS, a non- 3.2.3. Echocardiography
contrast series of images is typically obtained first, to Transthoracic Echocardiography (TTE)
better distinguish IMH, if present, from other causes of TTE is the most common imaging modality used in
aortic wall thickening. Then, a series of arterial phase the initial nonemergency assessment of the thoracic
contrast-enhanced images is obtained with thin slice to aorta.1,2 TTE is particularly useful in imaging the aortic
allow for reconstructions (computed tomographic angi- root and ascending aorta and in delineating aortic valve
ography [CTA]), extending from the thoracic inlet to the anatomy and function. Although not ideal for imaging
level of the femoral arteries, to define the full extent of of the aortic arch, TTE often does visualize the aortic
any dissection and thereby guide therapy. For consis- arch branch vessels and the proximal descending aorta
tency in this document, CT is used to refer to computed and can aid in diagnosis of coarctation of the aorta
tomography modality broadly, with specific imaging (CoA) and patent ductus arteriosus. TTE is portable and
techniques chosen dependent on a given clinical indica- can be performed at the bedside with a high spatial and
tion and patient history. temporal resolution. It can be useful in the evaluation
of patients with AAS to detect complications, including
3.2.2. Magnetic Resonance Imaging aortic valve regurgitation, left ventricular dysfunction,
MRI provides coverage of the entire aorta and branch and cardiac tamponade. TTE is useful in the longitu-
vessels, can characterize aortic wall changes in the setting dinal surveillance of aortic root and ascending
aortic dilation, provided those aortic segments are well ultrasound requires an operator who is familiar with both
visualized. the acquisition and interpretation of images.
Transesophageal Echocardiography (TEE)
3.2.5. Abdominal Ultrasound
TEE provides high-resolution images of most of the
thoracic aorta, apart from a short segment of the distal Vascular ultrasound is an effective and rapid imaging
ascending aorta just proximal to the innominate artery, modality and is the recommended diagnostic tool in
attributable to acoustic shadowing from the trachea. TEE screening for and surveillance of AAA. 1-3 The ultrasonic
is also very useful in detailing aortic valve anatomy and criterion for AAA is a diameter >3.0 cm, using primarily
function. TEE is particularly useful in the intraoperative the outer-edge to outer-edge measurement convention in
evaluation of patients with AAS in guiding both operative the anterior-posterior or transverse view.4-6 The sensi-
and endovascular repair strategies and the assessment of tivity of ultrasound to detect the presence of an aneurysm
true and false lumen flows before and immediately after approaches 100%,7 although interobserver variability ex-
aortic repair.1,2 ists, and successful imaging can be limited by obesity and
superimposed bowel gas.8
3.2.4. Intravascular Ultrasound Using B-mode imaging, color Doppler, and spectral
Intravascular ultrasound is an endovascular technology waveform analysis, a comprehensive ultrasound evalua-
designed to provide high-resolution intraluminal imaging tion of the abdominal aorta can quickly detect other aortic
1
of localized arterial and venous disease. Intravascular pathologies, such as plaque or mobile atheroma forma-
ultrasound is particularly useful in guiding the endovas- tion, arterial stenoses, mural thrombus, inflammation,
cular management of complex pathologies of the thoracic dissection, pseudoaneurysm, contained rupture, and
and abdominal aorta, because it reveals aortic size, tor- aortocaval fistulae, and these findings may prompt the
tuosity, plaque burden, calcification, branch vessel ostia, need for further imaging with CT or MRI. Abdominal ul-
and intravascular filling defects (eg, thrombus, dissection trasound can also be used for surveillance of patients who
flap), in addition to permitting landing zone assessment. 1 have undergone endovascular repair of AAA (EVAR); it
Such intravascular ultrasound imaging data may help to can detect aneurysm sac expansion, which may indicate
identify patients for whom endovascular treatment is the presence of an endoleak (Figure 11), defined as
high-risk or contraindicated. Intravascular ultrasound is abnormal flow outside of the aortic endograft, a finding
especially useful in the setting of aortic dissection 2-4 to that typically warrants confirmation by CT. The use of
distinguish true and false lumen anatomy and thereby contrast-enhanced color duplex ultrasound has shown
guide endovascular or open repair. Intravascular ultra- promising results in enhanced sensitivity in detection of
sound may be used to guide deployment of endovascular endoleaks, 9 although its use requires ongoing study.
stents and, during final assessment, to reduce the volume 4. MULTIDISCIPLINARY AORTIC TEAMS
of iodinated contrast used.5 Importantly, intravascular
Recommendations for Multidisciplinary Aortic Teams
1. For patients with acute aortic disease that requires urgent repair, a multidisciplinary team should
1 C-EO determine the most suitable intervention.
2. For patients who are asymptomatic with extensive aortic disease, or who may benefit from complex open
2a C-LD and endovascular aortic repairs, or with multiple comorbidities for whom intervention is considered,
referral to a high-volume center (performing at least 30-40 aortic procedures annually) with experienced
surgeons in a Multidisciplinary Aortic Team is reasonable to optimize treatment outcomes. 1-6
Synopsis high concentration of expertise in the evaluation and

Evidence-based standards for medical and surgical con- management of aortic disease, in which care is delivered in
ditions recognize the critical relationship among both a comprehensive, multidisciplinary manner. 7 The concept
hospital and surgeon case volumes and patient outcomes. of comprehensive heart valve centers was formally codified
Clinical excellence is further enhanced by collaborative, in the “2020 ACC/AHA Guideline for the Management of the
multispecialty teams to foster the best treatment of pa- Patient With Valvular Heart Disease,” 8 which emphasized
tients, especially for complex presentations with multi- the numerous essential components of such centers,
organ threats. Although there is no agreed on definition of a ranging from physician expertise, experience, and tech-
Multidisciplinary Aortic Team, an appropriate framework nical skill to data collection, research, and education, to
might be: A specialized hospital team with an exceptionally institutional facilities and resources. Although the specific
F I G U R E 1 4 Observed Relationship Between Annual Institutional Case Volume and Risk-Adjusted Odds Ratio for Operative Mortality 2 Standard
Deviations as Assessed With Regression Analysis
The odds ratio for operative mortality decreased as institutional case volume increased. Adapted from Hughes et al.4 Copyright 2013, with permission from
Elsevier Inc.
components of such teams may differ from center to cen- More recently, we have seen the rise in multidisciplinary
ter, the most common features that distinguish Multidis- heart teams focused on the care of patients with com-
ciplinary Aortic Teams include: Having cardiac surgical, plex coronary artery disease and patients with complex
vascular surgical, and endovascular specialists with heart valve disease; indeed, the important role of
extensive experience managing complex aortic disease at a multidisciplinary heart valve teams was emphasized in
center with a high volume of aortic interventions; having the “2020 ACC/AHA Guideline for the Management of
imaging specialists with expertise in aortic disease to the Patient With Valvular Heart Disease.” 8 There is
perform and interpret CT, MRI, and echocardiography; ample evidence that patients with complex aortic dis-
anesthesiologists experienced in the management of acute ease may similarly benefit from treatment by such
aortic disease and cerebrospinal fluid drainage; and an multidisciplinary teams.6 Andersen et al1 compared the
intensive care unit (ICU) experienced in the management outcomes of patients with acute type A aortic dissection
of acute aortic disease. undergoing open surgical repair before and after
implementation of a multidisciplinary thoracic aortic
surgery program and found that operative mortality
1. In cardiovascular care, we have long recognized the declined dramatically after implementation of the
critical value of collaborative multidisciplinary exper- multidisciplinary team and that the significant mortality
tise in cardiac transplantation and mechanical circula- advantage persisted over a 5-year follow-up (P¼0.002).
tory support conducted only at centers of excellence. Likewise, in a report from England, 2 hospitals with
F I G U R E 1 5 Predicted Risk of Mortality Derived From the Logistic Regression Model Without Center Case Volume as a Covariate
Actual mortality and the ratio of actual mortality to predicted mortality (A/P ratio, the risk-adjusted mortality rate) are also shown. A similar predicted risk of
mortality across the case volume strata and a decrease in the actual mortality at higher center case volume are seen. Reprinted from Mori et al.9 Copyright 2018,
with permission from Elsevier Inc.
multidisciplinary thoracic aortic programs reported patients with acute aortic dissection. In a retrospective
significant reductions in mortality compared with hos- review of 232 patients with acute type A aortic dissec-
pitals without such programs. tion who underwent urgent surgery in a single center in
2. In a study of 230,736 Medicare beneficiaries undergo- the United Kingdom, the 30-day mortality rate was
ing AAA repair between 2001 and 2006, in which hos- significantly lower among those operated on by a sur-
pital procedural volume for both open and geon with aortic expertise versus a nonaortic expert, at
endovascular repair was divided into quintiles, the 10% versus 26%, respectively (P¼0.02). Moreover,
adjusted mortality decreased as hospital volume aortic specialists performed aortic root procedures
increased, by quintile, especially among the group significantly more often (43.0% versus 17.3%;
undergoing open surgical repair.3 The benefits of high P¼0.001), and their cross-clamp times were signifi-
case volume on surgical outcome apply similarly to cantly shorter. 5 Finally, Umana-Pizano et al10 found
patients with TAA. Hughes et al 4 analyzed >13,000 that the mortality rate of acute type A aortic dissection
elective aortic root and aortic valve-ascending aortic repair was 14% versus 24% for high-volume and low-
procedures performed at 741 North American hospitals volume surgeons, respectively. Clearly not all patients
from 2004 to 2007. They found a negative association with thoracic aortic disease (TAD) can be treated by
between the hospital volume and the adjusted odds Multidisciplinary Aortic Teams, especially in the
ratio (OR) for mortality (P<0.001), particularly at a setting of AAS. Nevertheless, when patients are
hospital volume of <30 to 40 procedures annually referred for elective aortic intervention, especially at
(Figure 14). The inverse relationship between center aortic diameter thresholds that are borderline, the
case volume and mortality was shown again in a more lower surgical mortality rate with expert aortic sur-
contemporary series by Mori et al 9 of >53,000 proximal geons at high-volume centers may justify early aortic
thoracic aortic surgeries in the United States from 2011 repair. Similarly, when aortic procedures are relatively
to 2016 in which the risk of operative mortality new or complex, the best outcomes are likely to be at
decreased significantly when the annual center volume centers with high-volume operators who have experi-
exceeded 20 to 25 cases (only 116 U.S. centers per- ence with such novel techniques. Consequently,
formed >20 cases/y), and decreased significantly throughout this guideline is a number of recommen-
further still at an annual center volume of >50 cases dations in which it is specified that certain open sur-
(only 24 U.S. centers performed >50 cases/y) gical or endovascular aortic repairs be performed by
(Figure 15). Perhaps the most consistent correlation experienced operators in centers with Multidisci-
between case volume and mortality rate is among plinary Aortic Teams.
5. SHARED DECISION-MAKING
Recommendations for Shared Decision-Making
1. In patients with aortic disease, shared decision-making is recommended when determining the appro-
1 C-LD priate thresholds for intervention, deciding on the type of surgical repair, choosing between open surgical
versus endovascular approaches; and in medical management and surveillance.1-6
2. In patients with aortic disease who are contemplating pregnancy or who are pregnant, shared decision-
1 C-EO making is recommended when considering the cardiovascular risks of pregnancy, the diameter thresholds
for prophylactic aortic surgery, and the mode of delivery.
Synopsis diameter threshold for prophylactic aortic repair, and

Shared decision-making is increasingly used in patient- the mode of delivery. This has particular relevance in
centered care as advocated by the National Academy of patients with Marfan syndrome and Loeys-Dietz syn-
Medicine.7 Although no randomized trials have evaluated drome, and other heritable aortic disorders who are
the value and effectiveness of shared decision-making, planning a pregnancy.
multiple position papers advocate strongly for the incor-
poration of shared decision-making in the care of patients
with thoracic and AAAs. 2-5 Decision aids have been 6. ANEURYSMS
developed for shared decision-making in patients with
AAAs to help improve the patient understanding of the 6.1. Thoracic Aortic Aneurysm (TAA) Causes
disease and treatment options.1 Shared decision-making TAAs occur in 5 to 10 per 100,000 person years. 1 The
is especially useful when considering the diameter natural history and treatment vary depending on the
thresholds for and the timing of intervention in addition cause and location of the TAA. The size of a given segment
to having an important role in considering the risks of of the thoracic aorta is influenced by age, sex, height, and
pregnancy in patients with underlying aortic disease. body size.2 Aortic z-scores and other diameter indexing
methods (see Section 2.3, “Definitions of Dilation and
Aneurysm of the Aortic Root and Ascending Thoracic
1. Shared decision-making is an active process in which Aorta”) may assist with risk assessment. 3 Of all TAA, an-
patients and families are encouraged to share their eurysms of the aortic root, ascending aorta, or both are
values and preferences regarding quality of life, goals most common (w60%), followed by those of the
of care, and desired procedural outcomes. Formally descending aorta (w30%) and arch (<10%). Hypertension,
recognizing those preferences helps physicians to bet- smoking, hypercholesterolemia, and heritable genetic
ter frame the risks and benefits of intervention versus variants are risk factors for TAA disease. Patients with
conservative management. Actively involving patients TAA have a modestly increased incidence of AAA 4 and
in the decision-making process is especially important cerebral aneurysms.5
in situations in which there is clinical equipoise, such Causes of TAA include heritable disorders, congenital
as: an aortic aneurysm with a diameter at the border- conditions, multifactorial degenerative conditions, pre-
line of the threshold for repair; performing valve- vious aortic dissection, inflammatory diseases, and in-
sparing root repair rather than valved-conduit aortic fectious diseases (Table 6). Aneurysms of the aortic root
root replacement; performing thoracic endovascular and ascending thoracic aorta tend to have a heritable in-
aortic repair (TEVAR) in a patient with an uncompli- fluence and present at younger ages, whereas aneurysms
cated type B aortic dissection who is at increased risk of of the descending thoracic aorta tend to be degenerative
complications; or treating an AAA with open surgical and present at older ages. 6 Moreover, aneurysms of the
versus endovascular repair. Shared decision-making aortic root and ascending thoracic aorta are also
may be used for noninterventional issues as well, commonly associated with BAV, although the genetic
such as the choice of medical therapies or the imaging basis of BAV and why some but not all patients have a
modality used for surveillance. concomitant aortopathy are not well understood. Finally,
2. Shared decision-making has an important role in many aneurysms of the root and ascending thoracic aorta
pregnancy among those with aortic disease to deter- are sporadic and idiopathic. Because the management of
mine whether to consider conception, an appropriate patients with aneurysms of the aortic root and ascending
TABLE 6 Cause of TAA
HTAD (see Table 7): syndromic

n Marfan syndrome
n Loeys-Dietz syndrome
n Vascular Ehlers-Danlos syndrome
n Smooth muscle dysfunction syndrome
n Others: attributable to pathogenic variants in FLNA, BGN, LOX
HTAD (see Table 7): nonsyndromic

n ACTA2, MYH11, PRKG1, MYLK, and others
n Familial thoracic aortic aneurysm without identified pathogenic variants in a known gene for HTAD
Congenital conditions
n Bicuspid aortic valve
n Turner syndrome
n Coarctation of the aorta
n Complex congenital heart defects (tetralogy of Fallot, transposition of the great vessels, truncus arteriosus)
Hypertension
Atherosclerosis
Degenerative
Previous aortic dissection
Inflammatory aortitis
n Giant cell arteritis
n Takayasu arteritis
n Behçet disease
n Immunoglobulin G4-related disease, antineutrophil cytoplasmic antibody-related, sarcoidosis
Infectious aortitis
n Bacterial, fungal, syphilitic
Previous traumatic aortic injury
HTAD indicates heritable thoracic aortic diseases; and TAA, thoracic aortic aneurysms.
F I G U R E 1 6 Recommendations for Management of Aneurysms of the Aortic Root and Ascending Aorta According to Known Causative Factors.
TABLE 7 TAA Syndromes and Conditions Attributable to a Heritable or Genetic Cause
Condition Gene Clinical Features
Syndromic HTAD*
Marfan syndrome FBN1 Aortic root aneurysm, aortic dissection, TAA, MVP, long bone overgrowth, arachnodactyly,
dolichostenomelia, scoliosis, pectus deformities, ectopia lentis, myopia, tall stature, pneumothorax,
dural ectasia
Loeys-Dietz syndrome TGFBR1, TGFBR2, SMAD3,† TAA, branch vessel aneurysms, aortic dissection, arterial tortuosity, MVP, craniosynostosis, hypertelorism,
TGFB2, TGFB3 bluish sclera, bifid/broad uvula, translucent skin, visible veins, club feet, dural ectasia, and premature
osteoarthritis and peripheral neuropathy†
Vascular Ehlers-Danlos syndrome COL3A1 TAA, AAA, arterial rupture, aortic dissection, MVP, bowel and uterine rupture, pneumothorax, translucent
skin, atrophic scars, small joint hypermobility, easy bruising, carotid-cavernous fistula
Arterial tortuosity syndrome SLC2A10 Tortuous large and medium sized arteries, aortic dilation, craniofacial, skin and skeletal features
Shprintzen-Goldberg syndrome SKI Craniosynostosis, skeletal features, aortic dilation
Ehlers-Danlos syndrome with FLNA X-linked, periventricular nodular heterotopia, TAA, BAV, MV disease, PDA, VSD, seizures, joint
periventricular nodular heterotopia hypermobility
Meester-Loeys syndrome BGN X-linked, TAA, aortic dissection, MV disease
LOX-related TAA LOX TAA, BAV, aortic dissection, Marfanoid habitus in some
Smooth muscle dysfunction syndrome ACTA2 TAA, moyamoya-like cerebrovascular disease, pulmonary hypertension, pulmonary disease,
hypoperistalsis, hypotonic bladder, congenital mydriasis11
Nonsyndromic HTAD (Familial TAA)
FTAA ACTA2 TAA, aortic dissection, premature CAD and moyamoya-like cerebrovascular disease, livedo reticularis, iris
flocculi
FTAA MYH11 TAA, aortic dissection, PDA
FTAA MYLK Aortic dissection at relatively small aortic size
FTAA PRKG1 Aortic dissection at young ages at small aortic sizes
FTAA MAT2A TAA, aortic dissection, BAV
FTAA MFAP5 TAA, aortic dissection, skeletal features may be present
FTAA FOXE3 TAA, aortic dissection
FTAA THSD4 TAA, aortic dissection
Bicuspid Aortic Valve–Associated Ascending Aortic Aneurysm
Familial BAV/AS and TAA NOTCH1 Aortic valve stenosis, TAA
BAV with TAA TGFBR2, MAT2A, GATA5, Syndromic and nonsyndromic HTAD and FTAA with an increased frequency of BAV
SMAD6, LOX, ROBO4, TBX20
Turner syndrome XO, Xp BAV, CoA, TAA, aortic dissection, short stature, lymphedema, webbed neck, premature ovarian failure
*Some individuals with pathogenic variants in a gene that can lead to syndromic HTAD have very few or no syndromic features, and variants in some genes causing syndromic HTAD may also lead
to nonsyndromic HTAD.
†SMAD3 premature osteoarthritis and peripheral neuropathy.
AAA indicates abdominal aortic aneurysm; AS, aortic stenosis; BAV, bicuspid aortic valve; CAD, coronary artery disease; CoA, coarctation of the aorta; EDS, Ehlers-Danlos syndrome; FTAA,
familial thoracic aortic aneurysm (and dissection) syndrome; HTAD, heritable thoracic aortic disease; MV, mitral valve; MVP, mitral valve prolapse; PDA, patent ductus arteriosus; TAA, thoracic
aortic aneurysm; and VSD, ventricular septal defect.
thoracic aorta may differ depending on the underlying aortic disease and aortic dissection. 8 Pathogenic variants
cause or family history, the recommendations for medical in multiple genes can lead to TAA, cerebral aneurysms,
and surgical therapy are grouped accordingly in the and AAA. 7,8 Up to 20% of individuals with a TAA or aortic
document, as shown in Figure 16. dissection have a family history of TAD, with at least 1
Approximately 20% of TAA are related to a genetic or affected first-degree relative.8 Population studies have
heritable condition (also referred to as heritable thoracic shown the familial nature of TAAs and dissections, with
aortic disease [HTAD]), some of which associate with familial cases having a significantly increased risk of TAA
multisystem features (considered syndromic HTAD) and and aortic dissection 8,9 compared with sporadic cases.
others with abnormalities limited to the aorta with or Therefore, among patients with aortic root and ascending
without its branches (known as nonsyndromic HTAD) 7 aortic aneurysm or those with aortic dissection, screening
(Table 7). HTAD most commonly involves the aortic root, of first-degree relatives with imaging is essential to detect
ascending aorta, or both but may also present with distal unrecognized, asymptomatic TAD. 8,10
6.1.1. Sporadic and Degenerative TAA descending aorta, at 8% to 9% versus 80% to 88%,
Although there is a well-recognized anatomic distinction respectively.1 Collectively, these findings suggest that
between aneurysms of the thoracic versus abdominal aneurysms of the aortic root and ascending aortic tend to
aorta, this should not imply that all TAA are similar in have a congenital if not hereditary cause, whereas aneu-
cause or natural history. Aneurysms of the aortic root and rysms of the descending aorta tend to have an athero-
ascending aorta are typically diagnosed at younger pa- sclerotic cause. Although sometimes referred to as
tient ages than aneurysms of the descending thoracic atherosclerotic aneurysms, more often aneurysms of
aorta (60 versus 72 years, respectively).1 Even when descending thoracic aorta (not related to connective tis-
considering just the “sporadic” aneurysms (ie, aneurysms sue disorders) are referred to as “degenerative.” The
in which there is no evidence of a syndromic, familial, or medical management and surgical and endovascular
known genetic etiology), a significant difference in the management of sporadic and degenerative aneurysms are
ages between the 2 groups (64 versus 72 years, respec- discussed in Sections 6.4, “Medical Management of Spo-
tively) persists.1 In addition, typical atherosclerosis risk radic and Degenerative Aortic Aneurysm Disease,” and
factors (ie, hypertension, diabetes, smoking) are signifi- 6.5, “Surgical and Endovascular Management of Aortic
cantly less common in sporadic root and ascending versus Aneurysms,” respectively.
descending aortic aneurysms. 2 Moreover, the prevalence
6.1.2. Genetic Aortopathies
of aortic calcification or atheroma (by CT or MRI) is quite
6.1.2.1. HTAD: Genetic Testing and Screening of
low in sporadic aneurysms of the root and ascending
Family Members for TAD
thoracic aorta but quite high in aneurysms of the
Recommendations for HTAD: Genetic Testing and Screening of Family Members for TAD
1. In patients with aortic root/ascending aortic aneurysms or aortic dissection, obtaining a multigenerational
1 B-NR family history of TAD, unexplained sudden deaths, and peripheral and intracranial aneurysms is recom-
mended. 1-3
2. In patients with aortic root/ascending aortic aneurysms or aortic dissection and risk factors for HTAD
1 B-NR (Table 8, Figure 17), genetic testing to identify pathogenic/likely pathogenic variants (ie, mutations) is
recommended. 4-6
3. In patients with an established pathogenic or likely pathogenic variant in a gene predisposing to HTAD, it
1 B-NR is recommended that genetic counseling be provided and the patient’s clinical management be informed
by the specific gene and variant in the gene. 7-9
4. In patients with TAD who have a pathogenic/likely pathogenic variant, genetic testing of at-risk bio-
1 B-NR logical relatives (ie, cascade testing) is recommended.6,10,11 In family members who are found by genetic
screening to have inherited the pathogenic/likely pathogenic variant, aortic imaging with TTE (if aortic
root and ascending aorta are adequately visualized, otherwise with CT or MRI) is recommended. 4,5,12
5. In a family with aortic root/ascending aortic aneurysms or aortic dissection, if the disease-causing variant
1 B-NR is not identified with genetic testing, screening aortic imaging (as per recommendation 4) of at-risk
biological relatives (ie, cascade testing) is recommended. 13-15
6. In patients with aortic root/ascending aortic aneurysms or aortic dissection, in the absence of either a
1 C-LD known family history of TAD or pathogenic/likely pathogenic variant, screening aortic imaging (as per
recommendation 4) of first-degree relatives is recommended.13
7. In patients with acute type A aortic dissection, the diameter of the aortic root and ascending aorta should
1 C-EO be recorded in the operative note and medical record to inform the management of affected relatives.
TABLE 8 Risk Factors for Familial TAD

2. The HTAD genetic testing panels include (at the time of
this writing) 11 genes that are confirmed to confer a
TAD and syndromic features of Marfan syndrome, Loeys-Dietz syndrome, or
vascular Ehlers-Danlos syndrome highly penetrant risk for TAD: FBN1, LOX, COL3A1,
TAD presenting at age <60 y TGFBR1, TGFBR2, SMAD3, TGFB2, ACTA2, MYH11,
A family history of either TAD or peripheral/intracranial aneurysms in a first- or
MYLK, and PRKG1.19 These panels also include genes
second-degree relative that increase the risk for TAD and/or lead to systemic
A history of unexplained sudden death at a relatively young age in a first- or features that overlap with Marfan syndrome, Loeys-
second-degree relative
Dietz syndrome, or vascular Ehlers-Danlos syndrome.
TAD indicates thoracic aortic disease. Clinical genetic testing is integral to the diagnostic
evaluation of patients with TAD who have clinical in-
Synopsis dicators suggestive of an underlying single gene dis-
A major risk factor for aortic root aneurysms, ascending order (Table 8).5,20 In patients who meet the clinical
aortic aneurysms, and aortic dissection is a pathogenic diagnostic criteria for Marfan syndrome but do not
variant in genes predisposing to TAD. Although the rec- have ectopia lentis (ie, dislocated lens), genetic testing
ommendations focus on individuals at high risk for a is reasonable to exclude an alternative diagnosis of
single gene mutation (Table 8), genetic testing may have a Loeys-Dietz syndrome. Genetic testing laboratories
role in many TAD patients. A multigene panel comprising categorize rare variants in HTAD genes into these
all genes suspected to cause HTAD is the most cost- classes: pathogenic, likely pathogenic, variant of un-
effective and clinically useful approach to testing. Only certain/unknown significance, benign, and likely
pathogenic or likely pathogenic variants are disease- benign. Variants of unknown significance have not
causing and should be used for cascade genetic testing been confirmed to cause TAD and therefore should not
all relatives at risk for inheriting the disease-causing be used either to identify which family members are at
variant.15,16 risk or to guide clinical management. Because a subset
In families with HTAD in which the causative gene has of these variants of unknown significance may,
not been identified, the clinical features in affected family nevertheless, be disease-causing, families with the
member should dictate management of other family potential to help further classify the variant of un-
members, including location of aneurysms; relevant known significance should be evaluated in collabora-
clinical features include the diameter of the aortic root tion with the genetic testing company.
and ascending aorta in affected family members who have 3. FBN1, TGFBR1, TGFBR2, SMAD3, and TGFB2 mutations
had a type A dissection (noting that the aortic root typi- have been identified in approximately 6% to 8% of
cally is not distorted by the dissection, whereas the HTAD families whose members do not have syndromic
ascending aorta may acutely enlarge17) and other vascular features of Marfan syndrome or Loeys-Dietz syn-
disease or features segregating with TAA in the family. drome. 12,20-23 Mutations in ACTA2, MYH11, MYLK, LOX,
and PRKG1 have been confirmed to cause HTAD in the
absence of significant features of Marfan syndrome or
1. Current data indicate that 13% to 20% of patients with Loeys-Dietz syndrome.16,24 Through clinical charac-
TAD and without Marfan syndrome or Loeys-Dietz terization of HTAD families with pathogenic variants in
syndrome features have similarly affected first-degree novel genes, data have emerged that the underlying
relatives. 1,2 TAD in these families is typically inheri- gene predicts not only who in the family is at risk for
ted in an autosomal dominant manner, with decreased thoracic aortic aneurysm and dissection (TAAD) but
penetrance, particularly in women. These data suggest also the aortic disease presentation, risk for aortic
that heterozygous pathogenic variants in single genes dissection at a given range of aortic diameters as
are responsible for HTAD in most families. 3,18 In fam- described previously, and risk for and type of addi-
ilies with HTAD, testing in an individual diagnosed tional vascular diseases.7-9 For example, TGFBR2 mu-
with TAD should be initiated. Patients with a family tations predispose to TAAD but also to intracranial
history of the disease present at younger ages (average aneurysms and aneurysms and dissections of other
57 years). 3 These families with HTAD show variable arteries, whereas ACTA2 mutations lead to TAAD and
expression of TAD, including varying age of disease occlusive vascular disease, including early onset stroke
onset, frequency of aortic dissection at a diameter <5.0 and coronary artery disease. Genetic counseling is
cm, risk for type B aortic dissection, and frequency useful to explain to patients and families the genetic
with which dilation involves the aortic root, the risk and how it is inherited, to assess the family history
tubular ascending aorta, or both. 8,14 In addition, the to determine TAD risk, to assist in cascade genetic
specific altered gene impacts the risk for associated testing and/or imaging for TAD in family members, and
vascular conditions. to offer psychosocial and ethical guidance. 10
F I G U R E 1 7 Evaluation and Genetic Testing Protocol for Patients With TAD
Genetic testing is recommended for individuals with syndromic features, family history of TAD, and/or early age of disease onset. Thoracic aortic imaging is recom-
mended for first-degree relatives of all individuals with TAD, regardless of age of onset, to detect asymptomatic aneurysms. Positive genetic testing should trigger
gene-based management and cascade testing of at-risk relatives. When testing is negative or reveals variants of unknown significance, first-degree relatives should
undergo screening aortic imaging. Modified with permission from Milewicz et al.6 Copyright 2021, Minerva Medica. Blue (þ) indicates positive; green (–), negative; LDS,
Loeys-Dietz syndrome; MFS, Marfan syndrome; TAAD, thoracic aortic aneurysm and dissection; TAD, thoracic aortic disease; and VUS, variants of unknown significance.
*Aneurysms are typically asymptomatic.
4. Cascade screening is the process of extending imaging the ascending aorta), current aortic diameter or diam-
to identify asymptomatic thoracic aortic enlargement eter at the time of surgical repair or diameter at the
to individuals at risk within a family for inheriting the time of type A aortic dissection, and the presence of
pathogenic variant causing HTAD in the family; the other vascular diseases (eg, aneurysms in other ar-
process is repeated as family members are identified teries, early onset occlusive vascular diseases), as these
with thoracic aortic enlargement or as carriers of the will inform management of all affected family mem-
pathogenic variant are identified. 10 Pathogenic vari- bers. The HTADs vary in terms of the risk of other
ants in genes for HTAD confer a high risk for TAD, so clinical cardiovascular complications that segregate
individuals found to have these pathogenic variants with TAD; therefore, surveillance for such conditions is
should be screened with aortic imaging for asymp- best guided by the family history. 22,25-27
16,24
tomatic TAD. 6. Although the data are more limited, studies also sup-
5. Among patients undergoing genetic testing, many will port the screening of first-degree relatives of patients
not have a pathogenic variant identified, despite other with TAD who do not have a family history of the dis-
clinical evidence that the disease is likely genetically ease. 13 If negative, aortic imaging may be repeated
triggered (eg, extensive family history of TAD or early years later, depending on the relative’s age and aortic
onset sporadic TAD with no risk factors). Despite the size. It should be recognized that there is no upper
absence of a pathogenic variant among the currently limit to the age at which patients present with TAD that
known genes that were tested, TAD could still be precludes an underlying genetic cause of the disease.
inherited in the family attributable to a causative ge- 7. Because the size at which the aortic root or ascending
netic variant that has yet to be identified. Conse- aorta dissects impacts the risk of aortic dissection in
quently, multiple studies have confirmed the utility of other affected family members, the specific aortic di-
screening aortic imaging of at-risk relatives of all TAD ameters should be recorded in the medical record (ie,
patients with a positive family history. 13-15 If negative, operative report, discharge summary), so that the in-
repeat screening imaging might be worthwhile in 5 formation can be readily retrieved when needed in the
years of younger family members or 10 years in older future.
family members, informed by the family history.
Additionally, it is critical to obtain relevant clinical
6.1.2.1.1. Surgical Considerations for Nonsyndromic Heritable
data from affected family members, including the
TAAs and No Identified Genetic Cause
location of the aortic dilation (ie., the aortic root versus
Recommendations for Surgical Considerations for Nonsyndromic Heritable TAA and No Identified Genetic Cause
1. In asymptomatic patients with aneurysms of the aortic root or ascending aorta with nonsyndromic her-
1 C-LD itable thoracic aortic disease (nsHTAD) and no identified genetic cause, determining the timing of surgical
repair requires shared decision-making and is informed by known aortic diameters at the time of aortic
dissection, TAA repair, or both in affected family members. 1-4
2. In asymptomatic patients with aneurysms of the aortic root or ascending aorta with nsHTAD and no
1 C-LD identified genetic cause but no information on aortic diameters at the time of dissection or aneurysm
repair in affected family members and who have no high-risk features for adverse aortic events (Table 9)
it is recommended to repair the aorta when the maximal diameter reaches ‡5.0 cm.1
3. In patients with aneurysms of the aortic root or ascending aorta with nsHTAD and no identified genetic
2a C-LD cause and a maximal aortic diameter of ‡4.5 cm, who have high-risk features for adverse aortic events
(Table 9), or who are undergoing cardiac surgery for other indications, aortic repair is reasonable when
performed by experienced surgeons in a Multidisciplinary Aortic Team. 5
Features Associated With an Increased Risk of

than for sporadic aneurysms; aneurysm growth is also
TABLE 9 Aortic Dissection in Patients With Heritable typically faster than for sporadic aneurysms. Because the
Thoracic Aortic Aneurysms initial presentation is commonly acute aortic dissection,
Heritable Thoracic Aortic Aneurysms and No Identified Genetic Cause screening family members is important to guide prophy-
Family history of aortic dissection at an aortic diameter <5.0 cm lactic surgery to prevent potential aortic dissection.
Family history of unexplained sudden death at age <50 y

Clearly, elective surgery before aortic dissection yields
better long-term survival with fewer aortic reinterven-
Rapid aortic growth ($0.5 cm in 1 y or $0.3 cm/y in 2 consecutive y)
tions than surgery after aortic dissection.4-7
Synopsis 1. The GenTAC (National Registry of Genetically Trig-

HTAD refers to TAD caused by a highly penetrant rare gered Thoracic Aortic Aneurysms and Cardiovascular
variant (or mutation) in a single gene. A diagnosis of Conditions) study found a higher risk of dissection,
HTAD is based on $2 members of a family with TAD, with most of dissection patients not having met the
the identification of a pathogenic variant in the gene size criteria for prophylactic surgery.1,6 For patients
known to cause TAD in a family member, or clinical with a family history of TAA, aortic dissection, or both,
diagnosis of syndrome that confers a risk for TAD (eg, but with no known pathogenic variant, it is useful to
Marfan syndrome) in a family member. Syndromic determine the size at which the aorta dissected (if
HTAD typically has systemic features with multiorgan known) or the size at which elective aortic surgery was
phenotype, positive family history of aortic aneurysm or performed, as well as the age of the affected relative at
dissection, and is often caused by mutations involving time of the aortic event. It is appropriate to offer aortic
extracellular matrix proteins or involved in trans- repair based on the family member’s aortic size at
forming growth factor-b pathway. Such patients, dissection or elective surgery.
including Marfan syndrome and Loeys-Dietz syndrome, 2. Patients with a family history of TAAs but with no
are predisposed to developing aneurysms of the aortic known pathogenic variant may not have information
root and ascending aorta at an early age, and have a regarding the aneurysm size at which the family
faster rate of aortic growth than do those with sporadic members underwent either elective surgery or experi-
aneurysms. Consequently, these patients have a higher enced aortic dissection. However, the GenTAC study
risk of acute aortic dissection or rupture, resulting in a suggested a higher risk of aortic dissection, with a large
shorter life expectancy than those patients whose an- proportion of patients not having met the 5.5-cm
eurysms are not genetically mediated. Prophylactic threshold for elective repair at the time of their aortic
surgery to replace the aortic root and ascending aorta dissection. Given that aortic dissection in this popula-
has dramatically improved the overall life expectancy of tion with familial TAAs may occur at younger ages and
HTAD patients. Prophylactic elective surgery in these with worse outcomes and the more frequent need for
young patients requires a very low operative mortality reoperations, prophylactic surgery is warranted when
with a multidisciplinary approach for genetic testing the maximal diameter of the aortic root or ascending
and lifelong surveillance. Surgeons in Multidisciplinary aorta reaches $5.0 cm.1-4,8
Aortic Teams have shown sufficiently low operative 3. For patients with a family history of aortic dissection at
mortality to safely treat these patients at smaller aortic a known maximal aortic root or ascending aortic
sizes. Similar to what is seen with sporadic aneurysms, diameter <5.0 cm but with no known pathogenic
aortic dissection in HTAD can occur at aortic diameters variant, it is reasonable to perform prophylactic aortic
smaller than the surgical thresholds recommended in repair at a maximal aortic diameter of $4.5 cm, because
guidelines. their affected relative experienced an aortic dissection
nsHTAD refers to a genetic predisposition to TAD at the relatively small diameter of <5.0 cm. Similarly,
running in families in the absence of systemic features. patients with relatives whose aortic dissection or un-
NsHTAD may be present in up to 20% of patients with explained sudden death occurred at an age <50 years
TAD (based on family history), is typically inherited in an are themselves at increased risk of such adverse events
autosomal dominant manner, with a pathogenic genetic at ages <50 years as well. Similarly, nsHTAD patients
variant identified in up to 20%. When no pathogenic who have documented rapid aneurysm growth are
variant is identified in families with nsHTAD, it has often increased risk of untoward aortic events at younger
been referred to as “familial thoracic aortic aneurysm and ages and smaller aneurysm sizes, so prophylactic aortic
dissection.” It tends to be more penetrant and of earlier surgery is reasonable when performed by experienced
onset in men than women within affected families. The surgeons in Multidisciplinary Aortic Teams, with
diagnosis is often delayed until midlife but occurs earlier shown excellent short- and long-term outcomes.1-4,8
6.1.2.2. Marfan Syndrome

6.1.2.2.1. Diagnostic and Surveillance Aortic Imaging in Marfan
Syndrome
Recommendations for Diagnostic and Surveillance Aortic Imaging in Marfan Syndrome

Initial Diagnosis and Surveillance Imaging
1. In patients with Marfan syndrome, a TTE is recommended at the time of initial diagnosis, to determine the
1 C-EO diameters of the aortic root and ascending aorta, and 6 months thereafter, to determine the rate of aortic
growth; if the aortic diameters are stable, an annual surveillance TTE is recommended. 1
If the aortic root, ascending aorta, or both are not adequately visualized on TTE, a CT or MRI of the
thoracic aorta is recommended.2
2. In adults with Marfan syndrome, after the initial TTE, a CT or MRI of the thoracic aorta is reasonable to
2a C-EO confirm the aortic diameters and assess the remainder of the thoracic aorta.
Imaging After Aortic Root Replacement
3. In patients with Marfan syndrome who have undergone aortic root replacement, surveillance imaging of
1 C-LD the thoracic aorta by MRI (or CT) is recommended to evaluate for distal TAD, initially annually and then, if
normal in diameter and unchanged after 2 years, every other year. 3-6
4. In patients with Marfan syndrome who have undergone aortic root replacement, surveillance imaging
2a C-LD every 3 to 5 years for potential AAA is reasonable.2,6
Synopsis but effacement of the sinotubular junction with

Marfan syndrome is an autosomal dominant connec- enlargement of the proximal ascending aorta is often
tive tissue disorder caused by pathogenic variants in the present. 11 The aortic root and ascending aorta are
1
FBN1 gene affecting 1 in 5,000 individuals. Phenotypic measured by TTE and are observed annually. Nomo-
features in the skeletal, ocular, pulmonary, cutaneous, grams accounting for age, sex, and body size (and
nervous, and cardiovascular systems may be recognized. height) assist with determining the degree to which the
The modified Ghent criteria for diagnosis incorporate diameter deviates from normal in the general popula-
genetic testing, the systemic score, ectopia lentis, and the tion. 12 In patients with Marfan syndrome participating
1
family history. Patients with Marfan syndrome develop in trials of beta blockers versus angiotensin receptor
aneurysms involving the aortic root (sinuses of Valsalva) blockers (ARBs), the mean growth of the aortic root was
and are at risk for aortic dissection.1 Descending aortic 1 mm to 1.5 mm over 3 years and 4 mm to 5 mm over 5
and AAAs are less common. 6,7 Type B aortic dissection is years. The rate of aortic dilation is faster in patients
the initial aortic event in about 10% of patients and may with larger aortic aneurysms. More frequent imaging is
also occur despite previous root replacement.4 Imaging performed in patients with rapid aortic growth, in
surveillance of the aorta is typically performed annually, those approaching surgical thresholds, or when the
with the frequency dependent on age, aortic diameter, diameter exceeds 4.5 cm. Patients with Marfan syn-
rate of aortic growth, and family history. 8 Prophylactic drome are at greatest risk for aneurysmal dilation of
aortic root replacement for aneurysm disease prevents the aortic root, followed by involvement of the
type A dissection and improves survival in Marfan ascending aorta. Patient-specific factors, such as pec-
syndrome. 3,9,10 tus deformities and lung disease, may limit the evalu-
ation of the aortic root on TTE. When the aortic root
and ascending aorta are not adequately visualized by
1. Aortic root dilation and type A aortic dissection are the TTE, CT or MRI should be performed to measure the
leading causes of morbidity and mortality in Marfan aortic diameters, 2 although TEE is another alternative
9,10
syndrome. Aortic dilation involves the aortic root, to measure the aortic root and ascending aorta.
2. Patients with Marfan syndrome may develop disease of Long-term complications after aortic root replacement
the descending aorta. 9,10 In some individuals, a thor- may include graft infections, pseudoaneurysms, an-
ough TTE may accurately assess the diameters of aortic eurysms in the distal aorta, and aortic dissection distal
root, ascending aorta, aortic arch, proximal descending to the graft. 4,13
aorta, and distal descending aorta. For patients un- 4. In patients with Marfan syndrome, distal TAA and AAA
dergoing an initial evaluation in whom the aortic seg- (in the absence of aortic dissection) may occur but are
ments distal to the ascending aorta are not adequately much less common than aortic root disease. Most in-
visualized on TTE, a CT or MRI can be used to assess dividuals with aortic disease distal to the root have had
the more distal aortic segments. previous root replacement or smoke cigarettes. 7,13
3. Surgical aortic root replacement can prevent type A
aortic dissection and improve longevity for patients
6.1.2.2.2. Medical Therapy in Marfan Syndrome
with Marfan syndrome and aortic root aneurysms. 3,9,10
Recommendations for Medical Therapy in Marfan Syndrome

1. In patients with Marfan syndrome, treatment with either a beta blocker or an ARB, in maximally tolerated
1 A doses (unless contraindicated), is recommended to reduce the rate of aortic dilation. 1,2
2. In patients with Marfan syndrome, the use of both a beta blocker and an ARB, in maximally tolerated
2a C-LD doses (unless contraindicated), is reasonable to reduce the rate of aortic dilation. 3,4
Synopsis study of children with Marfan syndrome who had pre-

Beta blockers have long been recommended for patients viously had rapid aortic root growth, ARBs were shown
with Marfan syndrome to reduce heart rate and myocar- to dramatically slow aortic root growth. 10 However,
5-7
dial contractility and to slow aortic root growth. More randomized trials comparing an ARB to a beta blocker in
recently, ARBs have also been found to be efficacious in patients with Marfan syndrome found no significant
Marfan syndrome. 1-4,8 difference in the rate of either aortic root growth or
clinical events (including aortic surgery or aortic
dissection) between the 2 treatment groups. 1,2
1. In an open-label study of patients with Marfan syn- 2. Multiple trials have compared the addition of an ARB to
drome who were observed for >10 years, propranolol beta-blocker therapy in patients with Marfan syn-
treatment was associated with a reduction in aortic root drome 3,4,8; in 2 studies, the addition of an ARB led to a
5
growth rate and fewer clinical events compared with reduction of aortic root growth rates over a 3- to 5-year
control (no treatment). More recently, in a retrospective follow-up,3,4 and a meta-analysis confirmed slower
evaluation of children with Marfan syndrome, beta- aortic growth rates with combination therapy.11
blocker treatment was associated with a reduced
aortic growth rate. 6 Losartan was shown to prevent 6.1.2.2.3. Marfan Syndrome Interventions: Replacement of the
aneurysm formation in mouse models of Marfan syn- Aortic Root in Patients With Marfan Syndrome
drome 9 and, in a small, nonrandomized open label
Recommendations for Marfan Syndrome Interventions: Replacement of the Aortic Root in Patients With Marfan Syndrome
1. In patients with Marfan syndrome and an aortic root diameter of ‡5.0 cm, surgery to replace the aortic
1 B-NR root and ascending aorta is recommended. 1-4
2. In patients with Marfan syndrome, an aortic root diameter of ‡4.5 cm, and features associated with an
2a B-NR increased risk of aortic dissection (Table 10), surgery to replace the aortic root and ascending aorta is
reasonable, when performed by experienced surgeons in a Multidisciplinary Aortic Team. 1,3,4
(Continued)
3. In patients with Marfan syndrome and a maximal cross-sectional aortic root area (cm 2) to patient height
2a C-LD (m) ratio of ‡10, surgery to replace the aortic root and ascending aorta is reasonable, when performed by
experienced surgeons in a Multidisciplinary Aortic Team. 5
4. In patients with Marfan syndrome and an aortic diameter approaching surgical threshold, who are can-
2b C-LD didates for valve-sparing root replacement (VSRR) and have a very low surgical risk, surgery to replace
the aortic root and ascending aorta may be reasonable when performed by experienced surgeons in a
Multidisciplinary Aortic Team.2-4
Synopsis syndrome, aortic growth rates are relatively slow, but

Prophylactic aortic root replacement for aneurysm the growth rate increases with aortic size.12
disease prevents type A aortic dissection and improves 3. Aortic diameters vary depending on age, sex, height,
survival in Marfan syndrome. 6-8 The size threshold for and body size. Aortic event rates, including aortic
elective surgery to replace the dilated aortic root in Mar- dissection, increase as the aortic size indexed to height
fan syndrome is dependent on many factors, including (or body size) increases. When the maximal cross-
the patient’s age, height and weight, family history, rate sectional area in square (cm 2) of the aortic root or
1,3-5,9
of aortic growth, and other patient-specific factors. ascending aorta divided by the patient’s height (m) is
In patients with Marfan syndrome who are managed $10 cm2 /m, prophylactic aortic root replacement is
with optimal medical therapy and whose aortic diameters reasonable; when this cross-sectional area to height
are <5.0 cm, the risk of aortic dissection is low. 3,4,10 ratio was used to guide prophylactic surgery, patients
However, the risk of aortic dissection increases when had favorable outcomes.
the aortic diameter is >5.0 cm and is greater in patients 4. Aortic root replacement is associated with a very low
with a family history of aortic dissection or rapid aortic surgical risk3,4,11 when performed by experienced sur-
3,4,10
growth. geons in Multidisciplinary Aortic Teams. The 2 aortic
root replacement procedures performed most
Recommendation-Specific Supportive Text commonly in the United States are a composite valved
1. In patients with Marfan syndrome and a dilated aortic graft conduit and a VSRR.13 The composite valved graft
root, elective aortic root and ascending aortic replace- conduit consists of a prosthetic aortic valve (typically
ment before aortic dissection improves survival. 6-8 A mechanical but may be bioprosthetic) and aortic graft,
landmark report in 1995 documented the marked with reimplantation of the coronary arteries (often
improvement in lifespan among patients with Marfan referred to as the modified Bentall procedure). The
syndrome treated with elective aortic repair compared VSRR uses the David procedure, in which the native
with historical controls from previous eras. 6,10 aortic valve is reimplanted into a prosthetic aortic graft
Although risk of aortic dissection is low in patients that is attached to the left ventricular outflow tract
with Marfan syndrome who are receiving appropriate proximally and to the ascending aorta distally. The
medical care and lifestyle modifications, the risk of advantage of the VSRR is that, if successful, patients
aortic dissection increases when the aortic diameter is can potentially avoid the lifelong risks and complica-
>5.0 cm.3,4,11 When prophylactic surgical aortic repair tions associated with prosthetic valves. Consequently,
is performed, both the aortic root and ascending aorta early prophylactic surgery can be considered when
are replaced; although some centers have advocated both the procedural and late risks are low. However,
including hemiarch replacement in patients at the time durability of the spared native aortic valve is a poten-
of elective root/ascending aorta replacement, data to tial concern; in one series of 239 patients with Marfan
support this approach are lacking. syndrome undergoing VSRR, 7% developed at least
2. In large series of patients with Marfan syndrome, about moderate AR at 1 year follow-up. 13
20% have undergone elective surgery when aortic root
diameters are <5.0 cm.3,4,11 Predictors of aortic
dissection and other adverse aortic outcomes in Marfan Features Associated With Increased Risk of
TABLE 10
Aortic Complications in Marfan Syndrome
syndrome are listed in Table 10. Indications for earlier
aortic surgery may include rapid aortic growth ($0.3 n Family history of aortic dissection
n Rapid aortic growth ($0.3 cm/y)
cm/y), family history of aortic dissection, desire for
n Diffuse aortic root and ascending aortic dilation 14
pregnancy, severe valve regurgitation, and patient
n Marked vertebral arterial tortuosity 15
preference.3,9,12 For most patients with Marfan
6.1.2.2.4. Marfan Syndrome Interventions: Replacement of

Primary (Nondissected) Aneurysms of the Aortic Arch,
Descending, and Abdominal Aorta in Patients With
Marfan Syndrome
Recommendation for Replacement of Primary (Nondissected) Aneurysms of the Aortic Arch, Descending, and
Abdominal Aorta in Patients With Marfan Syndrome
COR LOE RECOMMENDATION
1. In patients with Marfan syndrome and a nondissected aneurysm of the aortic arch, descending thoracic
2a C-EO aorta, or abdominal aorta of ‡5.0 cm, surgical intervention to replace the aneurysmal segment is
reasonable.
Synopsis dissection involving these segments. 5 In patients at

Marfan syndrome most commonly leads to aneurysms acceptable risk for operative repair or with a long life
of the aortic root and ascending aorta but may also affect expectancy, operative intervention to resect primary
the distal aorta and its branches. 1-4 Unfortunately, there (nondissected) aneurysms involving the arch,
are no large datasets to inform the risk of aortic dissection descending, or abdominal aorta is reasonable at an
or rupture in patients with Marfan syndrome with pri- aortic diameter threshold of $5.0 cm, depending on the
mary (nondissected) aneurysms of the aortic arch, patient’s age, rate of aortic growth, family history, and
descending, or abdominal aorta, so using a 5.0-cm diam- surgical risk. Type B aortic dissection occurs in about
eter threshold for surgery, as is used for the aortic root, is 10% of Marfan patients, often in the absence of signif-
reasonable. icant dilation of the descending aorta, and is sometimes
associated with prior elective aortic root replacement, 1
a previous aortic dissection elsewhere, 6 or pregnancy.7
1. Although uncommon, aortic segments distal to the
aortic root and ascending aorta may dilate in Marfan
6.1.2.3. Loeys-Dietz Syndrome
syndrome, and this occurs more often after elective
aortic root replacement or after a previous aortic
6.1.2.3.1. Imaging in Loeys-Dietz Syndrome
Recommendations for Imaging in Loeys-Dietz Syndrome
1. In patients with Loeys-Dietz syndrome, a baseline TTE is recommended to determine the diameters of the
1 C-EO aortic root and ascending aorta, and 6 months thereafter to determine the rate of aortic growth; if the
aortic diameters are stable, annual surveillance TTE is recommended.1-3
2. In patients with Loeys-Dietz syndrome and a dilated or dissected aorta and/or arterial branches at
1 C-EO baseline, annual surveillance imaging of the affected aorta and arteries with MRI or CT is recommended.1
3. In patients with Loeys-Dietz syndrome, a baseline MRI or CT from head to pelvis is recommended to
1 C-LD evaluate the entire aorta and its branches for aneurysm, dissection, and tortuosity. 1-4
4. In patients with Loeys-Dietz syndrome without dilation of the aorta distal to the aortic root or ascending
2a C-EO aorta and without dilated or dissected arterial branches, surveillance imaging from chest to pelvis with
MRI (or CT) every 2 years is reasonable, but imaging may be more frequent depending on family history.
5. In patients with Loeys-Dietz syndrome without dilation of the cerebral arteries on initial screening, pe-
2a C-EO riodic imaging surveillance for cerebral aneurysms with MRI or CT every 2 to 3 years is reasonable.
Synopsis 2. Patients with Loeys-Dietz syndrome may have variable

Loeys-Dietz syndrome is characterized by aortic and aortic and branch vessel involvement and variable
branch vessel aneurysms and dissections, arterial tortu- rates of dilation of involved arterial segments over
osity, and skeletal features similar to those seen in Marfan time. In Loeys-Dietz syndrome patients with aortic
syndrome but with unique craniofacial and cutaneous aneurysm or previous dissection, relatively rapid
features.1 Pathogenic variants in 5 genes cause Loeys- arterial enlargement may occur.2,18,19
Dietz syndrome, also termed transforming growth factor- 3. Patients with Loeys-Dietz syndrome are at risk for
b vasculopathies.1-3,5,6 Some pathogenic variants in Loeys- widespread aortic and branch vessel aneurysmal dis-
Dietz syndrome genes, in particular TGFBR1 and TGFBR2, ease and dissections. 1,12 In a series of 90 patients with
7
may have earlier onset TAD. All the Loeys-Dietz syn- Loeys-Dietz syndrome attributable to pathogenic vari-
drome genes confer a risk for aortic involvement distal to ants in TGFBR1 and TGFBR2, aneurysm disease
the aortic root along with branch vessel and intracranial involved the ascending aorta in 78%, arch in 10%,
aneurysms.1,8-11 Most clinical information is available in descending aorta in 10%, abdominal aorta and
patients with TGFBR1 and TGFBR2 pathogenic variants.1,8 branches in 17%, thoracic aortic branches in 21%, and
Pathogenic variants in SMAD3 are associated with pre- head and neck arterial branches in 10%. 1 Among pa-
9,12
mature osteoarthritis and later onset of TAD. There is tients with SMAD3-related disease, aneurysms from
much less information about the aortic and branch vessel head to pelvis are also described.9,12 Individuals with
13-16
disease in patients with variants in TGFB2 and TGFB3. Loeys-Dietz syndrome can be at risk for TAD and other
Imaging with CT or MRI, from head to pelvis, is indicated vascular diseases in the absence of other systemic
to evaluate for aneurysms and arterial tortuosity. features characteristic of Marfan syndrome or Loeys-
Recommendation-Specific Supportive Text Dietz syndrome. Although there is phenotypic overlap
among the genes, there is also distinct vascular disease
1. Aortic root and ascending aortic aneurysm and aortic
and systemic complications associated with each gene.
dissection are leading causes of morbidity and mor-
1,8,9,12 The physician should be cognizant of the particular
tality in Loeys-Dietz syndrome. Aortic dissection
gene variant in monitoring and managing patients with
may occur at relatively small aortic diameters in Loeys-
Loeys-Dietz syndrome.
Dietz syndrome when related to pathogenic variants in
1,2,6 4. In patients with Loeys-Dietz syndrome and aortic dis-
TGFBR1, TGFBR2, and SMAD3. The specific genetic
ease limited to segments that are well-visualized by
variant and severity of extra-aortic phenotypic fea-
TTE and without branch vessel disease, surveillance of
tures, including craniofacial features, degree of arterial
the distal aorta and its branches is needed to evaluate
tortuosity, cutaneous findings, and family history
for the possible interval occurrence of dilation (or
inform the risk of aortic events. 1,2,6 The aortic root and
dissection); the frequency of surveillance imaging may
ascending aortic diameters are typically measured by
be influenced by the patient’s age and family history.2
TTE. BAV is more common in Loeys-Dietz syndrome
5. Cerebral aneurysms are described in 10% to 18% of
and can be diagnosed by TTE.17 Patients with Loeys-
patients with Loeys-Dietz syndrome. 1,9,11,20 The fre-
Dietz syndrome attributable to certain pathogenic
quency of follow-up screening for cerebral aneurysm
variants are at risk for aortic dissection at relatively
disease in patients without aneurysms on initial
small aortic diameters. 1,8 In patients with Loeys-Dietz
screening will depend on the patient’s age and may be
syndrome, the stability of the aortic size 6 months af-
informed by phenotype or other features.11
ter the initial diagnosis should be determined, and
then, once stability is confirmed, monitored with
annual surveillance imaging.1,2 6.1.2.3.2. Medical Therapy in Loeys-Dietz Syndrome
Recommendation for Medical Therapy in Loeys-Dietz Syndrome
1. In patients with Loeys-Dietz syndrome, treatment with a beta blocker or an ARB (unless contraindicated),
2a C-EO or both, in maximally tolerated doses, is reasonable.
Synopsis patients with Loeys-Dietz syndrome. Consequently, the

The management of individuals with Loeys-Dietz syn- approach to medical therapy is similar to that used for
drome includes medical therapy, lifestyle modification, treating patients with Marfan syndrome, based on the
imaging surveillance, and surgical intervention. To lessen similarities between the 2 connective tissue disorders
hemodynamic stress on the aorta, beta blockers are used. 1 and on data from mouse models of Loeys-Dietz syn-
Based on studies of mouse models, ARBs have also been drome.2 Thus, the use of beta blockers, ARBs, or both is
2
used. reasonable. 1
Recommendation-Specific Supportive Text 6.1.2.3.3. Loeys-Dietz Syndrome Surgical Interventions:

Replacement of the Aorta in Patients With
1. There are no randomized trials of medications to
Loeys-Dietz Syndrome
reduce aortic growth or the risk of aortic dissection in
Recommendations for Replacement of the Aorta in Patients With Loeys-Dietz Syndrome
1. In patients with Loeys-Dietz syndrome and aortic dilation, the surgical threshold for prophylactic aortic
1 C-LD root and ascending aortic replacement should be informed by the specific genetic variant, aortic diameter,
aortic growth rate, extra-aortic features, family history, patient age and sex, and physician and patient
preferences (Table 11). 1-9
2. In patients with Loeys-Dietz syndrome attributable to a pathogenic variant in TGFBR1, TGFBR2, or

2b C-EO SMAD3, surgery to replace the intact aortic arch, descending aorta, or abdominal aorta at a diameter
of ‡4.5 cm may be considered, with the specific genetic variant, patient age, aortic growth rate, family
history, presence of high-risk features (Table 11), and surgical risk informing the decision.
Synopsis pathogenic variants.1,2 Patients with TGFBR1 and

In patients with Loeys-Dietz syndrome, prophylactic TGFBR2 variants are at risk of type A aortic dissection at
aortic root replacement for aneurysm disease prevents younger ages and smaller aortic root diameters than in
type A aortic dissection and improves outcomes. 1,2,10-12 Marfan syndrome. 1,17,19 This aggressive aortopathy,
Aortic dissection in Loeys-Dietz syndrome that is attrib- especially in those with severe craniofacial features,
utable to pathogenic variants in TGFBR1, TGFBR2, and previously led to a recommendation for surgery at an
SMAD3 may occur at smaller aortic diameters than in aortic root diameter of >4.0 cm.1 The “2010 ACC/AHA
1-3,13
Marfan syndrome. Based on limited data, Loeys-Dietz Guidelines for the Management of Thoracic Aortic Dis-
syndrome attributable to pathogenic variants in ease” recommended aortic surgery at a diameter be-
TGFB2 5,6,14 and TGFB3 8,9 may have a less aggressive aortic tween 4.2 cm and 4.6 cm, depending on imaging
phenotype than disease attributable to TGFBR1, TGFBR2, modality.20 SMAD3-related Loeys-Dietz syndrome var-
1,2,4,15,16
or SMAD3 variants. The size threshold for elective iants may lead to aortic dissection at variable di-
surgery to replace the dilated aortic root and ascending ameters. 4,15,16,20 Aortic dissection risk is higher in
aorta in Loeys-Dietz syndrome depends on multiple fac- women with TGFBR2 variants who have certain extra-
tors and is informed by the specific pathogenic variant, aortic features.2 Limited data have not suggested higher
phenotypic features, patient age, aortic growth rates, and aortic dissection risk at smaller aortic size in those with
family history (Table 11).1,2,10-12,17 TGFB2 5,6,14 or TGFB3 variants. 8,9 Marked intrafamilial
There is little information about size thresholds for variability exists for aortic disease in Loeys-Dietz syn-
prophylactic surgery in Loeys-Dietz syndrome to lessen drome.17,21,22 A shared decision about timing of pro-
the risk of aortic dissection or rupture when there are phylactic surgery to prevent type A aortic dissection in
intact aneurysms involving the aortic arch, descending, or Loeys-Dietz syndrome should include consideration of
abdominal aorta, or involving aortic branch vessels. 11,12,18 the specific genetic variant, aortic diameter, aortic
After aortic dissection, progressive aneurysmal dilation growth rate, age, sex, body size, family history, patient
commonly occurs and often requires multiple operative preferences, and surgical expertise.
interventions.11,12,18
2. Aneurysms of the distal ascending aorta, arch,
descending aorta, and abdominal aorta may occur in
1. Pathogenic variants in TGFBR1, TGFBR2, SMAD3, Loeys-Dietz syndrome.1,2,5,8,9,11-14,16,17 At the time of
TGFB2, and TGFB3 lead to Loeys-Dietz syndrome or aortic root replacement, the entire ascending aorta is
may cause aortopathy with few outward features. Most also to be replaced because distal ascending aortic
information is available for TGFBR1 and TGFBR2 aneurysm and dissection may occur after isolated aortic
Surgical Thresholds for Prophylactic Aortic Root and Ascending Aortic Replacement in Loeys-Dietz Syndrome Based
TABLE 11
on Genetic Variant
COR LOE (references) Genetic Variant Presence of High-Risk Features* Aortic Diameter (cm)
1 C-LD2 TGFBR1 No ‡4.5
2
1 C-LD TGFBR2 No ‡4.5
2b C-EO2 TGFBR1 Yes ‡4.0
2a C-LD1,2 TGFBR2 Yes ‡4.0
2a C-EO13,16 SMAD3 – ‡4.5†
2b C-EO5-7 TGFB2‡ – ‡4.5†

9,23
2b C-EO TGFB3 – ‡5.0†
*Aortic surgery may be recommended at smaller aortic diameters in Loeys-Dietz syndrome attributable to TGFBR1 and TGFBR2 pathogenic variants when there are features that
associate with a higher risk of aortic dissection, including: certain specific pathogenic variants; women with TGFBR2 and small body size; severe extra-aortic features (ie, craniosy-
nostosis, cleft palate, hypertelorism, bifid uvula, marked arterial tortuosity, widened scars, and translucent skin); family history of aortic dissection (especially at young age or relatively
small aortic diameter); and aortic growth rate >0.3 cm/y.
†Family history, age, and aortic growth rate also inform surgical thresholds.
‡Pathogenic variants in the TGFB2 gene are different than variants in the TGFBR2 gene.
Colors correspond to COR and LOE in Table 2.
COR indicates class of recommendation; and LOE, level of evidence.
root replacement.10-12,19 There is little information (FDA) approval. In the absence of data showing efficacy in
about aortic size thresholds at which the risk of aortic vascular Ehlers-Danlos syndrome, other beta blockers are
dissection warrants elective surgery in the intact aortic often prescribed, with some physicians choosing alter-
arch, descending, or abdominal aorta in Loeys-Dietz native beta blockers with vasodilatory properties. There
syndrome. A shared decision should consider the are no studies showing a benefit of ARBs in vascular
pathogenic variant, aortic diameter, rate of aortic Ehlers-Danlos syndrome.
growth, age, sex, body size, patient preference, and the Surgical repair in vascular Ehlers-Danlos syndrome
surgeon’s preference and surgical expertise. Aortic in- carries an increased risk because of vascular fragility and
terventions in Loeys-Dietz syndrome are especially associated bleeding complications.1-3,5 Rapid arterial
10-12
common after aortic dissection. aneurysm growth or the occurrence of dissection are in-
dications for treatment, 1-3,5 but no data are available to
6.1.2.4. Vascular Ehlers-Danlos Syndrome: Imaging, guide diameter thresholds for prophylactic surgical
Medical Therapy, and Surgical Intervention intervention for aortic and arterial branch vessel aneu-
Vascular Ehlers-Danlos syndrome, affecting 1 in 50,000 to rysms in vascular Ehlers-Danlos syndrome.1-5 Conse-
100,000 individuals, is attributable to pathogenic variants quently, the decision to intervene for aortic and branch
in COL3A1 and leads to spontaneous aortic and arterial vessel aneurysms and dissections involves a Multidisci-
dissections, aneurysms, and rupture at young ages.1,2 The plinary Aortic Team and shared decision-making.3,6 Open
onset and severity of arterial pathology correlates with surgery requires meticulous technique to lessen vascular
the specific COL3A1 pathogenic variant.2 Imaging the and tissue trauma, and interventional techniques may
aorta and branches may identify arterial segments at involve arterial embolization and endovascular therapy,
risk, but the frequency of screening surveillance is depending on individual circumstances. 1,3,5
1-4
uncertain. Typical protocols include baseline MRI or CT Guidelines for management of pregnancy in vascular
from head to pelvis to evaluate the entire aorta and its Ehlers-Danlos syndrome are limited, given the lack of
branches, with annual surveillance imaging thereafter to data and the rarity of the condition. 9 The decision to
monitor any dilated or dissected aortic or arterial seg- proceed with pregnancy in vascular Ehlers-Danlos syn-
ments and imaging every 2 years when the initial imaging drome is complex; for some women with specific genetic
is normal.1,2,5 Notably, the aorta and arterial branches in variants, null mutations, and normal vascular imaging,
vascular Ehlers-Danlos syndrome may rupture (or dissect) the risk may be lower, but shared decision-making is
even without significant dilation.1-3 essential.9 Of 38 women with vascular Ehlers-Danlos
Medical therapy of vascular Ehlers-Danlos syndrome syndrome completing 82 deliveries, only 13% were
includes education, lifestyle modification, and avoidance aware of their diagnosis before pregnancy.9 Tissue
3,6
of invasive procedures when possible. Studies of cel- fragility complicates labor and delivery and poses risks for
iprolol, a beta blocker with vasodilatory properties, vascular events and wound complications.9,10 Complica-
have suggested a benefit in patients with vascular tions may occur after vaginal or cesarean deliveries, but
Ehlers-Danlos syndrome,7,8 but data were considered to most women known to have vascular Ehlers-Danlos syn-
be insufficient for US Food and Drug Administration drome undergo cesarean delivery. 9-12
6.1.2.5. Turner Syndrome
Recommendations for Diagnostic Testing, Surveillance, and Surgical Intervention for Aortic Dilation in Turner Syndrome
1. In patients with Turner syndrome, TTE and cardiac MRI are recommended at the time of diagnosis to
1 B-NR evaluate for BAV, aortic root and ascending aortic dilation, aortic coarctation, and other congenital heart
defects.1-9
2. In patients with Turner syndrome who are ‡15 years old, the use of the ASI (ratio of aortic diameter [cm]
1 B-NR to BSA [m2]) is recommended to define the degree of aortic dilation and assess the risk of aortic
dissection. 9,10,11
3. In patients with Turner syndrome without risk factors for aortic dissection (Table 12), surveillance im-
1 C-LD aging with TTE or MRI to evaluate the aorta is recommended every 5 years in children and every 10 years
in adults, as well as before planning a pregnancy.9,10,11
4. In patients with Turner syndrome and an ASI >2.3 cm/m2, surveillance imaging of the aorta is recom-
1 C-EO mended at least annually. 9
5. In patients with Turner syndrome and risk factors for aortic dissection (Table 12), surveillance aortic
1 C-EO imaging at an interval depending on the aortic diameter, ASI, and aortic growth rate is recommended
(Figure 18). 9
6. In patients with Turnery syndrome who are ‡15 years old and have an ASI of ‡2.5 cm/m2 plus risk factors
2a C-LD for aortic dissection (Table 12), surgical intervention to replace the aortic root, ascending aorta, or both is
reasonable.9,10
In those without risk factors for aortic dissection, surgical intervention to replace the aortic root, ascending
2b C-EO aorta, or both may be considered.
Synopsis Recommendation-Specific Supportive Text

Turner syndrome, which affects 1 in 2,500 liveborn
1. Turner syndrome may be recognized in infancy or
girls, results from complete or partial loss of the second X
childhood or, alternatively, go unrecognized until
chromosome in all or some of the cells of an individual. 9,12
adolescence or adulthood. On the diagnosis of Turner
Approximately 50% of patients with Turner syndrome
syndrome, a TTE and cardiac MRI are performed to
have cardiovascular defects that include BAV (15%-30%),
evaluate for associated congenital cardiovascular ab-
aortic coarctation (7%-18%), and ascending aortic dilation
normalities (BAV, aortic coarctation, and others) and to
(33%). 9,12 Patients with Turner syndrome require cardiac
measure aortic diameters.9,12
imaging to evaluate for congenital heart and aortic de-
fects and to determine aortic diameters. Patients with 2. Because patients with Turner syndrome have short
Turner syndrome are at increased risk of aortic dissection, stature, using absolute aortic diameters alone may
with 85% occurring in the ascending and 15% in the underestimate aortic dissection risk.9-11,13 Type A aortic
10,11,13
descending aorta. Risk factors for aortic dissection dissection in Turner syndrome may occur at relatively
include aortic dilation, hypertension, BAV, and aortic small aortic diameters, likely reflecting the typical pa-
coarctation.9-11,13 Because Turner syndrome patients are tient’s short stature, so indexing of aortic diameter to
of short stature, type A aortic dissection may occur at body size (by calculating the ASI) is performed when
relatively small aortic diameters; consequently, indexing evaluating patients with Turner syndrome who are $15
the aortic diameter to body size (ie, calculating an ASI) is years old.9,10 The ASI is calculated by dividing the
9,12,14
recommended in monitoring the aorta. maximal aortic diameter, in centimeters, by the BSA, in
additional risk factors for aortic dissection, surveil-

Risk Factors for Aortic Dissection in Patients
TABLE 12 lance imaging of the aorta with TTE or MRI every 10
With Turner Syndrome
years is appropriate.9 Surveillance imaging should also
n Aortic coarctation
n Aortic dilation
be performed before planned pregnancy.9
n Bicuspid aortic valve 4. In Turner syndrome, the risk of aortic dissection cor-
n Hypertension relates with ASI,9 and an ASI $2.5 cm/m2 is associated
with a significantly increased risk of aortic dissection.
meters squared. An ASI >2.0 cm/m 2 is considered to be When the ASI approaches this threshold, more
abnormal, and an ASI $2.5 cm/m2 is associated with an frequent surveillance imaging is appropriate to
increased risk of aortic disection. 9-11 Using a Turner monitor aortic diameters.9
syndrome-specific z-score to assess for aortic dilation 5. In Turner syndrome, risk factors for aortic dissection
is preferred in children <15 years old. include aortic dilation, BAV, aortic coarctation, and
3. Lifelong surveillance imaging of the aorta is used to hypertension.9-11,13 When these risk factors are present,
monitor for aortic dilation: For children with Turner surveillance imaging of the aorta is performed more
syndrome and no additional risk factors for aortic frequently. For the patients with Turner syndrome who
dissection, reevaluation at 5-year intervals is appro- are $15 years old and have a stable ASI of #2.3 cm/m 2,
priate; for adults with Turner syndrome and no surveillance imaging with TTE or MRI is performed
F I G U R E 1 8 Suggested Aortic Monitoring Protocol for Girls and Women With Turner Syndrome Who Are $15 Years of Age
*Surveillance frequency may vary depending on disease severity (ie, aortic valve dysfunction, severity of coarctation obstruction, hypertension, and left ventricular
hypertrophy).
Color corresponds to Class of Recommendations in Table 2.
ASI indicates aortic size index; BAV, bicuspid aortic valve; CoA, coarctation of the aorta; HTN, hypertension; MRI, magnetic resonance imaging; and TTE, transthoracic
echocardiography. Modified from Silberbach et al.9 Copyright 2018, with permission from American Heart Association, Inc. Modified from Gravholt et al.12 Copyright
2017, with permission from Bioscientifica Limited.
every 2 to 3 years.9 In the patients with Turner syn- nsHTADs, baseline imaging of the thoracic aorta with TTE,
drome who are >15 years old with an ASI >2.3 cm/m 2, at or with CT or MRI if the ascending aorta is not adequately
least annual surveillance imaging of the aorta is visualized by TTE, is recommended; surveillance imaging
appropriate. 9 The frequency of imaging should be is then performed annually, if stable. The arch and
informed by aortic diameter, aortic growth rate, descending aorta may dilate, in which case surveillance
severity of hypertension, and aortic valve function imaging of these segments is also performed. Less
(Figure 18). 9,12 frequent imaging may be considered when the aorta is
6. In patients with Turner syndrome, diameter thresholds normal, depending on gene variant, age, and family his-
for prophylactic surgical replacement of aneurysms of tory. Beta-blocker therapy is used to lessen hemodynamic
the aortic root/ascending aortic replacement are based stress on the aorta.
on retrospective series and case studies. 10,11,13 Data Specific features associated with each gene include:
from registries of aortic dissection in Turner syndrome Patients with ACTA2 mutations primarily present with
report that the risk of dissection is significantly type A or B aortic dissection, have aneurysms that
increased when the ASI is $2.5 cm/m 2.9-11,13 In addition involve the root and ascending aorta, and a subset of
to aortic size, risk factors for aortic dissection in Turner pathogenic variants predispose to occlusive vascular
syndrome include BAV, aortic coarctation, and hyper- diseases.2,5-7 Screening for coronary artery disease and
9,11,13
tension. However, decisions using indexed cal- cerebrovascular disease is performed in individuals with
culations alone for aortic risk determination in short- specific pathogenic variants. 5,6,8,9 Patients with ACTA2
statured but obese patients with Turner syndrome or mutations can suffer type A aortic dissection at aortic
those with low body weight relative to height may be diameters <4.5 cm, and consideration of surgery at
less accurate. In such Turner syndrome patients who diameters <4.5 cm is informed by the presence of addi-
are $15 years old, an absolute aortic diameter of >4.0 tional risk factors. 10 PRKG1-related HTAD can present in
cm may be more accurate than ASI in determining the the late teens with type A or B aortic dissection without
risk of aortic disection. 9 For patients with Turner syn- previous aortic enlargement11-13; patients with MYH11
drome who are <15 years old, a Turner syndrome- mutations primarily present with type A or B aortic
specific z-score calculation is appropriate to deter- dissection (type A aortic dissection may present at aortic
mine aortic risk and assess for surgical intervention. 9,14 diameters <5.0 cm), have aneurysms that involve the
For patients with Turner syndrome without additional root and ascending aorta, and may have peripheral
risk factors for aortic dissection, few data exist on the arterial disease 4,14 ; patients with MYLK mutations pre-
degree of aortic dilation that warrants surgical sent at age >40 years with type A aortic dissection with
intervention. 9 little previous enlargement of the aorta (median aortic
diameter, 4.25 cm)3,15,16; patients with LOX mutations
can present with aortic root aneurysms, fusiform dilation
6.1.2.6. Pathogenic or Likely Pathogenic Variants in ACTA2, of the root and ascending aorta that can extend into the
PRKG1, MYH11, MYLK, and LOX: Recommendations aortic arch, or type A aortic dissection, and they may
for Surveillance of Aorta, Medical Therapy, and have mild systemic features of Marfan syndrome.1,17,18
Aortic Surgical Intervention The decision regarding the timing of aortic repair in
Pathogenic variants in ACTA2, PRKG1, MYH11, MYLK, and nsHTAD is based on the aortic diameter, age, family
LOX confer a highly penetrant risk for TAD that is history, and the presence or absence of additional risk
inherited in an autosomal dominant manner.1-4 In these factors (Table 13).
Surgical Thresholds for Prophylactic Aortic Root and Ascending Aortic Replacement in Nonsyndromic Heritable
TABLE 13
Thoracic Aortic Disease Based on the Genetic Variant and Additional Risk Factors for Aortic Dissection
COR* LOE* Genetic Variant Risk Factors Aortic Diameter (cm)

2a C-LD ACTA2 No $4.5
2b C-EO ACTA2 Yes† $4.2
2b C-LD PRKG1 No $4.2
2b C-EO PRKG1 Yes† $4.0†
*Patient has risk factors for aortic dissection (family history of type A aortic dissection with minimal aortic enlargement, aortic growth rate $0.3 cm/y) or significant valve disease
requiring surgery.
†Earlier surgery may be considered in patients with a family history of type A aortic dissection in the setting of no or minimal aortic dilation, aortic growth rate $0.3 cm/y, or at the
patient’s request.
Colors correspond to COR and LOE in Table 2.
COR indicates class of recommendation; and LOE, level of evidence.
6.1.3. BAV Aortopathy
Recommendations for BAV Aortopathy

1. In patients with a BAV, TTE is indicated to evaluate valve morphology and function, to evaluate the
1 B-NR diameter of the aortic root and ascending aorta, and to evaluate for aortic coarctation and other
associated cardiovascular defects. 1-4
2. In patients with a BAV, CT or MRI of the thoracic aorta is indicated when the diameter and morphology of
1 C-LD the aortic root, ascending aorta, or both cannot be assessed accurately or completely by TTE. 1
3. In patients with a BAV and either HTAD or phenotypic features concerning for Loeys-Dietz syndrome, a
1 C-LD medical genetics evaluation is recommended.5,6
4. In patients with a BAV and a dilated aortic root or ascending aorta, screening of all first-degree relatives
1 C-LD by TTE is recommended to evaluate for the presence of a BAV, dilation of the aortic root and ascending
aorta, or both; if the diameter and morphology of the aortic root, ascending aorta, or both cannot be
assessed accurately or completely by TTE, a cardiac-gated CT or MRI of the thoracic aorta is indicated. 7
5. In patients with a BAV, screening of all first-degree relatives by TTE is reasonable to evaluate for the
2a B-NR presence of a BAV, dilation of the aortic root and ascending aorta, or both. 7-10
Synopsis aorta should be imaged by TTE to evaluate for

BAV is a common congenital valve condition affecting aortic valve function, aortic dilation, and aortic
approximately 1% of the population, with a 2 to 3:1 male- coarctation.1-4 Conversely, in other patients undergo-
3
to-female predominance. BAV most often occurs ing TTE, a finding of unexplained aortic root,
sporadically but may be inherited in an autosomal domi- ascending aortic dilation, or both should prompt sus-
nant pattern with variable penetrance. 5 A BAV may be picion of an underlying BAV15; if TTE of the aortic valve
isolated or associated with other congenital cardiovascu- is inconclusive for BAV, cardiac magnetic resonance,
lar defects or aortopathy conditions. 5 BAV is often asso- cardiac CTA, and TEE can be used to better visualize
ciated with aortic valve dysfunction (stenosis or the aortic valve and thereby diagnose BAV.
regurgitation) and is at risk of infective endocarditis. Pa- 2. Cardiac-gated CT or MRI provides superior images of
tients with a BAV often have aortic dilation or aneurysms the aortic root and ascending aorta when TTE is inade-
affecting the aortic root, ascending aorta, or both, with quate to visualize the full extent of the proximal aorta.
the prevalence of aortic aneurysm increasing with age.11 The choice between CT or MRI depends on patient
Distinct aortic dilation phenotypes have been characteristics, institutional expertise, renal function,
described.1,12 Those with BAV and a dilated aorta are at affordability, and radiation exposure concerns. 16
11,13,14
risk for type A aortic dissection. Patients with BAV 3. Certain types of HTAD have an increased prevalence of
require lifelong surveillance imaging of the aorta, even BAV. For example, BAV is present in w10% of patients
after AVR. with Loeys-Dietz syndrome (attributable to pathogenic
variants in TGFBR1, TGFBR2, SMAD3, TGFB2, and
TGFB3),6 and HTAD attributable to pathogenic variants
1. Aortic dilation in BAV may affect the aortic root, the in NOTCH1, ACTA2, MAT2A, SMAD6, and LOX also have
ascending aorta, or both. The ascending aorta is most an increased prevalence of BAV. 5,6 Importantly, most
commonly involved, and the dilation sometimes ex- patients with BAV and TAAs who undergo genetic
tends up into the arch. 1-3 The prevalence of aortic testing will not be found to have a pathologic genetic
dilation in BAV is reported from 20% to 84%, variant, even when their condition is familial. Never-
depending on the population studied and the defini- theless, when the condition is familial, a medical
tion of aortic dilation. 3,12 Patients with BAV and aortic geneticist or specialist in genetic aortopathy should
dilation are at risk for aortic dissection. 3,11,13 The aortic evaluate, counsel, and genetically test patients with
root, ascending aorta, arch, and proximal descending BAV and aortopathy.17
4. Both BAV and aortic root and ascending aortic dilation and aortic dilation may have members with aortic root
may be familial,7 and the inheritance patterns for fa- and ascending aortic dilation in the absence of a BAV, if
milial BAV and aortopathy are consistent with an the ascending aorta is not adequately assessed by TTE,
autosomal dominant pattern with incomplete pene- a CT or MRI should be performed to fully evaluate the
trance.8-10 In families with BAV and aortic root and size of the ascending aorta.
ascending aortic dilation, obligate carriers may have 5. The prevalence of a BAV in the relatives of a patient
BAV, aortic dilation, both, or neither.7 In families with with a BAV ranges from 9% to 20%. 8-10 Family mem-
BAV and aortic root and ascending aortic dilation, bers of individuals with a BAV may also have aortic
screening of the first-degree relatives (parents, sib- dilation. A recent analysis found that TTE screening of
lings, and children) with TTE to evaluate for BAV and first-degree relatives of affected patients, to detect
aortic dilation identifies affected members. If a family both BAV and aortopathy, proves to be cost-effective. 18
member is discovered to have a BAV, aortic dilation, or
both, cascade evaluation of other related family 6.1.3.1. Routine Follow-Up of BAV Disease Aortopathy
members is then indicated. Because families with BAV
Recommendations for Routine Follow-Up of BAV Disease Aortopathy

1. In patients with a BAV who have undergone previous aortic valve repair or replacement and have a
1 B-NR diameter of the aortic root, ascending aortic, or both of ‡4.0 cm, lifelong surveillance imaging of the
aortic root and ascending aorta by TTE, CT, or MRI is recommended at an interval dependent on aortic
diameter and rate of growth.1-3
2. In patients with a BAV and a diameter of the aortic root, ascending aorta, or both of ‡4.0 cm, lifelong
1 C-LD surveillance imaging of the aortic root and ascending aorta by TTE, CT, or MRI is recommended at an
interval dependent on aortic diameter and rate of growth. 4,5
Synopsis AR and those with predominant dilation of the aortic

Patients with BAV, with or without aortic dilation, root (“root phenotype”) are at higher risk for adverse
require lifelong surveillance of the aortic root and aortic events during follow-up. Among 56 patients
ascending aorta because of risk of late aortic growth. The with BAV who underwent isolated AVR for AR and
degree of aortic dilation and the progression of aortopathy had concomitant aortic root dilation (4.0–5.0 cm),
may be greater in patients with aortic root phenotype and adverse aortic events occurred in 34% of patients
those with predominant AR. 3,6 Progressive aortic growth during follow-up.4 Patients with BAV who undergo
3,7
may occur after AVR. isolated AVR for AR are at higher risk for late aortic
dissection than patients who underwent AVR for
aortic stenosis.10
1. Patients with BAV who have undergone previous 2. In a prospective study of 90 adults with BAV, the mean
isolated AVR or aortic valve repair remain at risk for increase in ascending aortic diameter was 0.47 mm/y
future aortic dilation and dissection. In a series of (range, 0.2–2.3 mm/y) over a 4.8-year follow-up.11 Sur-
1,286 patients who underwent isolated AVR for BAV veillance imaging can document current aortic di-
from 1960 to 1995, the 15-year freedom from aortic ameters and permit calculation of aortic growth rates. 2,6
events (aortic dissection, aortic aneurysm of >5.0 cm, Among a cohort of adult patients with BAV (mean age,
or aortic aneurysm surgery) was 89% but was lower 5517 years) without a TAA at baseline (ie, the baseline
for those with documented aortic dilation at baseline aortic diameter was <4.5 cm), 13% went on to develop a
compared with those with normal diameters (85% TAA at 146 years after diagnosis, and the 25-year risk
versus 93%; P¼0.001).8 Patients with BAV who have of TAA was 26%. 12 For many adults, an aortic root,
undergone isolated AVR for aortic stenosis and have ascending aortic, or both diameter $4.0 cm is consid-
only mild-to-moderate aortic dilation are at low risk ered dilated and should therefore be monitored over
for adverse aortic events at 15-year follow-up,3,9 time with surveillance imaging to detect progressive
whereas those who underwent AVR for predominant dilation.
6.1.3.2. BAV Aortopathy Interventions: Replacement of the

Aorta in Patients With BAV
Recommendations for BAV Aortopathy Interventions: Replacement of the Aorta in Patients With BAV
1. In patients with a BAV and a diameter of the aortic root, ascending aorta, or both of ‡5.5 cm, surgery to
1 B-NR replace the aortic root, ascending aorta, or both is recommended. 1-3
2. In patients with a BAV and a cross-sectional aortic root or ascending aortic area (cm2) to height (m) ratio
2a B-NR of ‡10 cm2/m, surgery to replace the aortic root, ascending aorta, or both is reasonable, when performed
by experienced surgeons in a Multidisciplinary Aortic Team. 3,4
3. In patients with a BAV, a diameter of the aortic root or ascending aorta of 5.0 cm to 5.4 cm, and an
2a B-NR additional risk factor for aortic dissection (Table 14), surgery to replace the aortic root, ascending aorta,
or both is reasonable, when performed by experienced surgeons in a Multidisciplinary Aortic Team.1,5
4. In patients with a BAV who are undergoing surgical aortic valve repair or replacement, and who have a
2a B-NR diameter of the aortic root or ascending aorta of ‡4.5 cm, concomitant replacement of the aortic root,
ascending aorta, or both is reasonable, when performed by experienced surgeons in a Multidisciplinary
Aortic Team. 1,6
5. In patients with a BAV, a diameter of the aortic root or ascending aorta of 5.0 cm to 5.4 cm, no other risk
2b B-NR factors for aortic dissection (Table 14), and at low surgical risk, surgery to replace the aortic root,
ascending aorta, or both may be reasonable, when performed by experienced surgeons in a Multidisci-
plinary Aortic Team. 1,2,5
Synopsis 3. There are additional risk factors for aortic

The timing of surgery to replace the aorta in BAV dis- dissection that may inform aortic surgical thresholds
ease depends on the morphology and diameter of the in patients with a BAV. A family history of aortic
10
aorta, aortic valve function, rate of aortic growth, family dissection and rapid aortic growth of $0.3 cm/y
history, patient characteristics, patient wishes, and the (when measured similarly with same technique)
expertise of the surgeon and institution.1,7 are both risk factors for aortic dissection. Patients
with BAV and aortic coarctation have been reported
to be at increased risk of aortic dissection,11 although
1. Patients with a BAV without significant aortic dilation in a recent report of 499 patients with BAV (mean
are at low risk for type A aortic dissection,3,8 whereas age, 4016 years), of whom 24% also had aortic
those patients with BAV and aneurysmal dilation of the coarctation, there was no difference in adverse aortic
aortic root, ascending aorta, or both have a signifi- events between those with or without coarctation.12
5,8
cantly increased risk of aortic dissection. The risk of Patients with dilation of the aortic root (“root
aortic dissection rises with increasing aortic diameter, phenotype”) represent 10% to 20% of patients
and there are “hinge points” when the ascending aorta with BAV and aortopathy and may have more rapid
reaches diameters >5.25 cm to 5.75 cm. 9 aortic growth and an increased risk of aortic
2. Indexing the maximal aortic root or ascending aortic complications. 13,14 Because surgical aortic root
diameter to height is predictive of aortic dissection risk replacement (and VSRR) is more complex than
and therefore informs surgical thresholds.3,4 Moreover, ascending aortic replacement, shared decision-
when comparing long-term outcomes in patients with making is often used when evaluating the risks and
BAV and aortic root or ascending aortic dilation, sur- benefits of elective aortic root replacement at aortic
vival was significantly better for those with an aortic diameters <5.5 cm.1,2,5,6
2
cross-sectional area (in cm ) to height (in meters) ratio 4. In patients with a BAV and indications for aortic valve
of $10 who underwent elective prophylactic aortic intervention for stenosis or regurgitation, the data are
repair compared with those who did not undergo limited regarding the degree of aortic dilation that
elective repair. 3 warrants replacement of the aortic root, ascending
TABLE 14 Risk Factors for Aortic Dissection

aortic stenosis and only moderate ascending aortic
dilation, the risks of concomitant aortic repair may not
Family history of aortic dissection
be warranted.
Aortic growth rate $0.3 cm/y
5. Limited data are available on the risk of aortic dissec-
Aortic coarctation
tion among those with a BAV and aortic aneurysm
“Root phenotype” aortopathy
diameter of 5.0 cm to 5.4 cm.5,15 Patient-related char-
acteristics and surgical expertise may inform the
timing of surgery, especially in low-risk patients with
aorta, or both at the time of AVR. Patients with a long BAV and aortic aneurysms of 5.0 cm to 5.4 cm.1,2,5,6
life expectancy, low surgical risk, or with the root
phenotype and predominant AR may benefit from
concomitant prophylactic aortic repair. Conversely, for
6.2. AAA: Cause, Risk Factors, and Screening
patients at higher surgical risk, especially those with
Recommendations for AAA: Cause, Risk Factors, and Screening

1. In men who are ‡65 years of age who have ever smoked, ultrasound screening for detection of AAA is
1 B-R recommended. 1
2. In men or women who are ‡65 years of age and who are first-degree relatives of patients with AAA,
1 C-LD ultrasound screening for detection of AAA is recommended.2,3
3. In women who are ‡65 years of age who have ever smoked, ultrasound screening for detection of AAA is
2a C-EO reasonable. 4,5
4. In men or women <65 years of age and who have multiple risk factors (Table 15) or a first-degree relative
2b C-LD with AAA, ultrasound screening for AAA may be considered. 5,6
5. In asymptomatic men or women >75 years who have had a negative initial ultrasound screen, repeat
3: No Benefit B-NR screening for detection of AAA is not recommended.1
Synopsis Takayasu Arteritis and Giant Cell Arteritis (GCA)”). The

Although AAA share risk factors with typical athero- growth of AAA is nonlinear, with a mean rate of 2.6 mm/y
sclerosis, AAA are histopathologically distinct and char- for AAA <5.0 cm,15 and may accelerate in the setting of
7
acterized by medial degeneration of the aortic wall. Most smoking or a family history of AAA, 16,17 and smoking may
AAA develop an intraluminal thrombus that contributes to have a greater impact on growth in women than in men.4
ongoing wall degradation via oxidative stress, smooth Ultrasound screening should be targeted toward those at
muscle cell apoptosis, proteolysis of the extracellular the greatest risk for AAA and growth (Figure 19), with the
matrix, and adventitial inflammation.8 A complex inter- goal of preventing rupture and associated mortality.1,5,6
play of hereditary and environmental risk factors con-
tributes to AAA, most notably older age, male sex,
smoking, and a positive family history (Table 15).2,3,9-12 1. Older age, male sex, and smoking are independent,
Lifetime risk for AAA is 8.2% in men and 10.5% in cur- strong risk factors for the development of AAA. 9-11
11
rent smokers. At least 10% to 25% of patients with AAA Smoking history is defined as lifetime use of $100 cig-
have a family member with the same condition, 2 and AAA arettes, but risk attributable to smoking varies signifi-
may occur concomitantly with thoracic aortic aneurysmal cantly depending on use, with lowest risk of AAA in
disease, especially in some genetic aortopathies. 11 In- those who have lower versus higher pack-year history. 9
flammatory aortitis is a rare cause of AAA 13,14 (Section 9.1, Based on a meta-analysis of randomized clinical trials
“Inflammatory Aortitis – Diagnosis and Treatment of inclusive of nearly 125,000 mostly male patients,
F I G U R E 1 9 Algorithm for Identifying Patients to Screen for Abdominal Aortic Aneurysm
Colors correspond to Class of Recommendations in Table 2. AAA indicates abdominal aortic aneurysm.
screening of men $65 years of age reduced long-term 2. Having a first-degree relative with AAA is a well-known
AAA-related mortality (4 RCTs: OR, 0.65; 95% CI, and well-established risk factor for development of
0.57–0.74) and AAA-related ruptures (4 RCTs: OR, 0.62; AAA.2,3 Small cohort studies of ultrasound screening in
1
95% CI, 0.55–0.70) over 12 to 15 years. In a recent relatives of those with AAA have identified an overall
population-based study (of both men and women) in prevalence of new AAA of 10% to 20%, with the highest
the United Kingdom, two-thirds of the acute AAA prevalence of 25% found among brothers. Indeed, the
events occurred in those $75 years of age; conse- overall lifetime prevalence of AAA is estimated to be
quently, screening to elderly patients should be 32% in brothers of those with AAA,2 suggesting the
offered, provided they would benefit from potential need for a targeted and individualized screening
aortic repair.18 approach for those who already meet age criteria
within families.
3. Select women may be at risk for AAA and related
complications. 5 Randomized trials and large observa-
TABLE 15 Risk Factors for Abdominal Aortic Aneurysm

tional studies that evaluate outcomes of screening for
AAA by ultrasound in women are lacking, as female sex
Strong Risk Factors Additional Risk Factors
has not been proven an independent risk factor for
Smoking history Hypertension
AAA,11 and overall prevalence of AAA in women is
Older age Hyperlipidemia lower than in men. However, the risk of AAA may be
Male sex White race potentiated by smoking in women; in 1 study, smoking
Family history of abdominal aortic Inherited vascular connective tissue was associated with a 15-fold increased risk of AAA
aneurysm disorder
among women (relative risk, 15.0; 95% CI, 13.2-17.0)
Atherosclerotic cardiovascular disease
versus 7-fold among men (relative risk, 7.3; 95% CI,
6.4-8.2).4 Practical implementation and outcomes of and the more common ascending aortic phenotype at 0.03
screening in women remain uncertain and warrant cm/y (0.3 mm/y).5 Moreover, among those with tricuspid
further study. aortic valves and sporadic ascending aortic dilation, the
4. Select patients <65 years of age may be at increased mean rate of growth is even slower, as low as 0.01 cm/y
risk of AAA rupture, and data suggest a significant (0.1 mm/y). 6 Aortic arch aneurysm growth has been re-
proportion of those undergoing repair for ruptured ported to be 0.25 cm/y. 7 The mean growth rate of
AAA did not meet the standard criteria for screening descending and TAAA has been reported to be 0.19 cm/y,
based on age.5,6 In a large study from the National with rates increasing as the diameter increases.8 The
Inpatient Sample, 10,603 of 25,777 patients with mean rate of growth of AAA is 0.26 cm/y, with larger an-
ruptured AAA (24%) were <65 years of age.5 Notably, in eurysms growing as fast as 0.5 cm/y. 9
patients <65 years, data are lacking on the mortality
benefit of AAA screening. 6.4. Medical Management of Sporadic and Degenerative Aortic
5. Some patients may develop AAA after the age of 75 Aneurysm Disease
years even if they had an initial negative screen be- The primary goals of medical therapy in sporadic and
tween the ages of 65 and 75 years. Although somewhat degenerative thoracic and abdominal aneurysmal disease
limited, data from cohort studies suggest long-term are to reduce growth rates, the risk of aortic-related
AAA-related mortality is low among patients with an mortality, and the need for aortic repair; a secondary
initial negative screening ultrasound who had a sub- goal is to decrease the risk of nonaortic cardiovascular
sequent AAA detected on repeat screening after the age events, given the multiple shared risk factors between
of 75 years.1 However, select patients at low surgical aneurysmal and atherosclerotic disease. 1,2 Lifestyle
risk who may have had borderline enlarged abdominal modification, including smoking cessation and blood
aorta measurements on initial screening and who have pressure (BP) control, improves overall cardiovascular
significant AAA risk factors (Table 15) may be consid- health and may be beneficial to patients with aortic
ered for repeat screening on an individualized basis. aneurysmal disease. Pharmacotherapy specific to the
treatment of aortic disease includes the use of selected
antihypertensives (especially beta blockers and ARBs)
that may mitigate the proteolysis pathways, leading to
6.3. Growth and Natural History of Aortic Aneurysms
medial degeneration and reducing of sheer stress on the
Aortic aneurysm growth and natural history is variable
aortic wall, as well as the use of statins, which may target
and dependent on the underlying etiology, such as HTAD
inflammatory and atherosclerotic pathways. 3 Outcomes
(eg, Marfan syndrome and Loeys-Dietz syndrome), BAV,
data from clinical trials of medical therapy in aortic an-
or sporadic aortic disease without a known genetic basis.
eurysms broadly are limited, as most trials have focused
There is significant evidence that aortic diameter corre-
on cohorts of patients with either Marfan syndrome or
lates with aortic dissection, aortic rupture, and mortal-
AAA. Consequently, correlations may be imprecise when
ity.1-3 In patients with Marfan syndrome, the mean rate of
applied to other populations.
growth of the aortic root has been reported to be 0.26 cm/
y (range, 0.13-0.35 cm/y), with a tendency for larger an- 6.4.1. Medical Therapy and Risk Factor Modification in
eurysms (>6.0 cm) to grow faster (0.46 cm/y). 4 Patients Sporadic TAA
with BAV have a slower rate of aortic growth, with a root
6.4.1.1. BP Management in Sporadic TAA
predominant phenotype growing at 0.06 cm/y (0.6 mm/y)
Recommendations for BP Management in TAA

1. In patients with TAA and an average systolic BP (SBP) of ‡130 mm Hg or an average diastolic BP (DBP)
1 B-NR of ‡80 mm Hg, the use of antihypertensive medications is recommended to reduce risk of cardiovascular
events.1-3
2. In patients with TAA, regardless of cause and in the absence of contraindications, use of beta blockers to
2a C-LD achieve target BP goals is reasonable.1,4,5
3. In patients with TAA, regardless of etiology and in the absence of contraindications, ARB therapy is a
2a C-EO reasonable adjunct to beta-blocker therapy to achieve target BP goals. 6
Synopsis diabetes over a median of 3.3 years, compared with a

The goal of BP control in TAA is to slow growth and control with a SBP target of <140 mm Hg.10,11
prevent aortic dissection, as well as to reduce nonaortic 2. Prospective data on the positive effects of beta blockers
cardiovascular events, such as myocardial infarction and in TAA based on cause are limited, with the most robust
stroke. Uncontrolled hypertension increases the risk for evidence derived from cohort studies of those with
aortic dissection,7 so achieving a SBP goal of #130 mm Hg Marfan syndrome (see Section 6.1.2.2, “Marfan Syn-
and a DBP goal of #80 mm Hg, with the use of antihy- drome”). In a small, open-label, randomized clinical
pertensive therapy in those with hypertension and TAA, trial of prophylactic propranolol (mean dose, 21268
is advised. Although data are limited, achieving a more mg/d) versus placebo in adolescents and adults with
intensive SBP goal of <120 mm Hg, if tolerated, may have Marfan syndrome, beta-adrenergic blocking drugs
added benefit in selected patients and who are not un- slowed aortic root growth and reduced aortic compli-
dergoing surgical repair.4 There has been significant cations. 5 In a study of 155 children <12 years of age with
progress in understanding the molecular basis of aneu- Marfan syndrome, beta blockers decreased the rate of
rysmal development and growth, 8 and a number of clin- aortic root growth by 0.16 mm/y, on multivariate
ical trials have explored the effects of beta-blocker and analysis. 4 In the “2017 Hypertension Clinical Practice
9
ARB therapy. A summary of these trials specific to ge- Guideline,” beta-blocker therapy is the recommended
netic aortopathies is covered in detail in Section 6.1.2, first-line antihypertensive drug therapy for patients
“Genetic Aortopathies.” However, as the molecular with hypertension and TAD. 1
mechanisms of aneurysm formation may have similarities 3. A meta-analysis of 1,510 randomized patients evalu-
between aneurysm patients with and without Marfan ating the effect of ARBs on TAA associated with Marfan
syndrome, data from these studies may be extrapolated in syndrome showed slower growth of the aortic root with
guiding the treatment of aortic disease of other causes. the use of ARBs compared with placebo; in a direct
Further clinical trials are clearly needed. comparison with beta-blocker therapy, there was no
difference in aortic growth; and the combination of
beta blocker plus ARB led to slower aortic growth than
1. No randomized clinical trials have evaluated the beta blockers alone.6 In the Jikei Heart Study,12 which
optimal threshold to which BP should be lowered in supported the use of ARBs in the 2010 ACC/AHA
patients with TAA to reduce the risk of aortic compli- thoracic aortic disease guidelines, Japanese patients on
cations (aortic growth, aortic dissection, or aortic an antihypertensive drug regimen that included val-
rupture). Updated hypertension guidelines from the sartan had a lower rate of adverse cardiovascular
ACC and AHA suggest all patients with clinical cardio- events, including mortality and, in particular, a
vascular disease should have a target SBP <130 mm Hg, reduction was showed in the incidence of aortic
DBP <80 mm Hg, or both. 1 Evidence supports aggres- dissection. However, this study was subsequently
sive BP lowering to reduce vascular-related adverse retracted 13 and, consequently, the LOE for use of ARBs
events and all-cause mortality.2,3 Data from SPRINT has been downgraded to C from B.
(Systolic Blood Pressure Intervention Trial) showed
that intensive BP control to a SBP <120 mm Hg, if
6.4.1.2. Treatment of TAA With Statins
tolerated, reduced cardiovascular events by 25% and
all-cause mortality rate by 27% in patients without
Recommendations for Treatment of TAA With Statins
1. In patients with TAA and imaging or clinical evidence of atherosclerosis, statin therapy at moderate or
2a C-LD high intensity is reasonable.1,2
2. In patients with TAA who have no evidence of atherosclerosis, the use of statin therapy may be
2b C-LD considered. 3-6
Synopsis RCTs of 39,612 patients over median 5.1 years, more

Clinical atherosclerotic cardiovascular disease (ASCVD) intensive cholesterol lowering in patients with ASCVD
encompasses aortic aneurysms of atherosclerotic origin. reduced major cardiovascular events by an additional
For the purpose of this guideline, we also define aortic 15% beyond what was achieved with less intensive
aneurysm with concomitant PAU or visualized atheroma cholesterol lowering.1,2 In patients with sporadic or
as atheromatous aortic disease, even in the presence of a genetically mediated aneurysms, if there is concomi-
genetic syndrome, given some causes have shared risk tant atherosclerotic disease elsewhere, then statin
factors with ASCVD. Based on the AHA/ACC “2018 therapy is still reasonable.
Guideline on the Management of Blood Cholesterol,” 1 a 2. It has long been hypothesized that the pleiotropic ef-
high-intensity statin for >50% reduction in low-density fects of statins may be beneficial in preventing the
lipoprotein (LDL) for patients <75 years of age with clin- adverse vascular wall remodeling associated with
ical atherosclerotic cardiovascular disease was recom- TAAs, thereby slowing growth, regardless of cause and
mended to prevent adverse events (eg, myocardial whether associated atherosclerosis is present. Animal
infarction and stroke). If a high-intensity statin cannot studies have shown a reduction in thoracic aneurysm
be used, a moderate-intensity statin can be initiated. 1 growth with statin therapy, possibly via regulation of
According to evidence from animal studies in MMP activity. 7,10 A study of 1,348 patients with
nonatherosclerotic-related TAA, statin therapy may pre- thoracic aortic ectasia showed, in a propensity-
vent growth and adverse remodeling 7. However, its use matched analysis, a possible benefit with statin ther-
in clinical practice at this time is not fully understood. apy in the reduction of aortic growth rate as well as
aortic complications. 3,11 In a retrospective study that
included 2,267 patients who underwent TEVAR for
1. Atherosclerotic aortic aneurysms increase risk of stroke aneurysmal disease, 1,148 (64%) of whom had been
and myocardial infarction and thus are considered a treated with a statin preoperatively, preoperative
coronary artery disease equivalent according to NCEP statin therapy was associated with significantly lower
ATP III (National Cholesterol Education Program Adult perioperative complication rates and 5-year mortal-
Treatment Panel III), with a >20% risk of an event ity. 12 A possible benefit of statins in prevention of
8
within 10 years. The “2016 AHA/ACC Guideline on the adverse aortic-related outcomes was also showed in a
Management of Patients With Lower Extremity Pe- small cohort study, and slowing of aortic growth is
ripheral Artery Disease” 9 gave a COR 2a recommenda- suggested by 2 small studies in patients with BAV and
tion for use of high-intensity statin in patients with aortopathy.6,8
noncoronary atherosclerotic disease to achieve an LDL
goal of <70 mg/dL. From the Cholesterol Treatment 6.4.1.3. Smoking Cessation in TAA
Trialists’ Collaboration, when combining data from 5
Recommendation for Smoking Cessation in TAA
1. In patients with TAA who smoke cigarettes, smoking cessation efforts are recommended. 1,2
1 C-LD
Synopsis been shown to be an effective strategy in smoking cessa-

Smoking cessation and avoidance of secondhand smoke tion,4 the efficacy and, importantly, safety of e-cigarette
exposure is considered a healthy lifestyle modification in use in patients with TAA is not well understood.
patients with TAAs, regardless of cause. Many patients
cared for in cardiovascular clinical practices have interest
in smoking cessation; thus, implementation of an effective 1. There are many validated options for smoking cessation
strategy using the 5 A’s (Ask, Advise, Assess, Assist, and for patients who continue to smoke and have TAA. 1-3
Arrange) is worthwhile, along with a referral to dedicated Although no randomized clinical trials have evaluated
programs, use of app-based tools, pharmacotherapy the effect of smoking cessation on outcomes in TAA,
(which includes nicotine replacement, bupropion, or var- smoking is a risk factor for TAA expansion and, among
enicline), or both.1-3 Although the use of e-cigarettes has those with atherosclerotic aortic disease, smoking
cessation reduces the rates of myocardial infarction effect on vascular endothelial function and relaxation
and death.5,6 The use of e-cigarettes, although an via nitric oxide and cyclic guanosine monophosphate-
effective smoking cessation tool, has not been shown to mediated signaling. 7,8
be safe when used in patients with vascular disease,
including TAA; further, small studies suggest that the 6.4.1.4. Antiplatelet Therapy in TAA
flavoring chemicals in e-cigarettes may have an adverse
Recommendation for Antiplatelet Therapy in TAA
1. In patients with atherosclerotic TAA and concomitant aortic atheroma or PAU, the use of low-dose aspirin is
2a C-EO reasonable, unless contraindicated.1,2

Aortic aneurysms of atherosclerotic origin are consid-
1. In the SPARC (Stroke Prevention: Assessment of Risk in
ered a coronary artery disease equivalent according to the
a Community) study, aortic atherosclerosis was associ-
NCEP ATP III, with a >20% risk of an event within 10
3 ated with coronary artery disease (OR, 2.99; 95% CI,
years. The 2006 updated “AHA/ACC Guidelines for Sec-
1.47–6.10; P¼0.003). 2 In turn, in the presence of coro-
ondary Prevention for Patients With Coronary and Other
nary artery disease, aspirin has long been recommended
Atherosclerotic Vascular Disease” 1 recommend use of low-
to reduce the risk of cardiovascular events, including
dose aspirin (75–162 mg/d) in patients with atherosclerotic
stroke, death caused by coronary artery disease, and
aortic disease. Even in the absence of TAA, this remains
myocardial infarction.1
true in other atherosclerotic aortic diseases, such as high-
grade atheroma, PAU, or both. 6.4.2. Medical Therapy and Risk Factor Modification in AAA
6.4.2.1. BP Management in AAA
Recommendation for BP Management in AAA

Referenced studies that support the recommendation are summarized in the Online Data Supplement.
1. In patients with AAA and an average SBP of ‡130 mm Hg, or an average DBP of ‡80 mm Hg, the use of
1 B-NR antihypertensive medication is recommended to reduce risk of cardiovascular events.1-3
Synopsis mm Hg.4 The most robust evidence of antihypertensive

Reducing cardiovascular events such as myocardial therapy in AAA is for beta blockers and agents that alter
infarction and stroke, as well as preventing aneurysm the renin angiotensin system; however, in prospective
growth and rupture, are the main goals in antihyperten- clinical trials in humans, no specific agent has been proven
sive therapy in AAA. Uncontrolled hypertension is a to inhibit AAA growth.
known risk factor for aortic rupture and dissection;
therefore, achieving an SBP goal of <130 mm Hg, and a
DBP goal of <80 mm Hg with the use of antihypertensive 1. Updated hypertension guidelines from the ACC and
therapy in those with hypertension and AAA can reduce AHA suggest all patients with clinical cardiovascular
adverse clinical outcomes, and some patients may benefit disease have a target SBP of <130 mm Hg and/or
from more intensive lowering with an SBP goal of <120 DBP <80 mm Hg. 1 Evidence supports aggressive BP
lowering to reduce vascular-related adverse events and reduced cardiovascular events by 25% and all-cause
all-cause mortality.2,3 A more intensive SBP goal mortality by 27% in patients without diabetes over a
of <120 mm Hg, if tolerated, may have added benefit in median of 3.3 years, compared with a control with an
select patients without diabetes and who are not un- SBP target of <140 mm Hg. 4,5
dergoing surgical aortic repair. However, data are
limited to the single randomized SPRINT,4 which 6.4.2.2. Treatment of AAA With Statins
showed that intensive BP control to SBP <120 mm Hg
Recommendations for Treatment of AAA With Statins

1. In patients with AAA and evidence of aortic atherosclerosis, statin therapy at moderate or high intensity is
1 B-NR recommended. 1-3
2. In patients with AAA but no evidence of atherosclerosis, statin therapy may be considered.4,5
2b C-LD
Synopsis current, but not a history of previous, statin use was

ASCVD includes noncoronary atherosclerotic disease associated with decreased 30-day mortality rates in
such as peripheral artery disease (PAD) and AAA. 6 For the patients with ruptured AAA (46.1% versus 59.3%,
purpose of this guideline, we define abdominal AAA of respectively; adjusted mortality rate, 0.80; 95% CI,
atherosclerotic cause as those with visualized aortic wall 0.68-0.95). 10 Retrospective data from 5,892 patients
atheroma, penetrating aortic ulceration either within the enrolled in the EUROSTAR (EUROpean collaborators on
aneurysm or at another site along the aorta, or both, with a Stent-graft Techniques for abdominal aortic Aneurysm
limitation being that many patients with genetically Repair) registry showed improved survival over 5 years
mediated AAA (see Section 6.2, “AAA: Cause, Risk Factors of follow-up associated with statin use (81% for statin
and Screening”) may have concomitant ASCVD. The AHA/ users versus 77% for nonusers; P¼0.005).11 Addition-
ACC “2018 Guideline on the Management of Blood ally, in a large registry-based study of 37,950 patients
Cholesterol,” 9 using evidence from the Cholesterol undergoing repair of AAAs, those not previously on
Treatment Trialists’ Collaboration, recommended a high- statin therapy who were started on statin before
intensity statin or, in some cases, moderate-intensity, for discharge had improved 1- and 5-year survival
patients with clinical ASCVD. A 50% reduction in LDL for compared with those who remained off statin therapy.12
such patients <75 years of age can prevent adverse events, 2. In a recent meta-analysis, in broad cohorts of patients
such as myocardial infarction and stroke. 7 Ongoing study with AAA, statin therapy was associated with slower
is needed to evaluate clinical outcomes of statin therapy aneurysm growth, reduced risk of rupture, and lower
in patients with nonatherosclerotic AAA. 30-day mortality after aortic repair 4 ; because athero-
sclerosis is so prevalent among patients with AAA, it
was not possible to distinguish whether statin therapy
1. AAA of atherosclerotic cause is considered a coronary benefited those without atherosclerosis equally. The
artery disease equivalent, with a >20% risk of a car- mechanisms by which statins improve survival in AAA
diovascular event within 10 years. 8 Intensive choles- warrant further study, as in 1 single prospective cohort
terol lowering in patients with ASCVD reduces major study of patients undergoing long-term surveillance,
cardiovascular events by an additional 15% beyond statins had not been shown to slow the growth rate of
what is achieved with less intensive cholesterol AAA or have direct effect on matrix metalloproteinase-9
lowering. 7,9 From a large Danish case-control study, or interleukin-6 concentrations. 5
6.4.2.3. Smoking Cessation in AAA
Recommendation for Smoking Cessation in AAA
1. In patients with AAA who smoke cigarettes, smoking cessation efforts are recommended.1-4
1 C-LD

Cigarette smoking is a major risk factor for the devel-
1. No randomized clinical trials have assessed the effect of
opment, growth, and complications of AAA (see Section
smoking cessation on clinical outcomes in patients with
6.2, “AAA: Cause, Risk Factors, and Screening”) and in-
AAA, given the inherent design limitations of such an
creases the risk for adverse clinical outcomes in the peri-
intervention. Current guidelines and recommendations
operative setting for AAA repair. Healthy lifestyle
that encourage counseling and pharmacological in-
modifications in ASCVD, such as atherosclerotic AAA and
terventions in patients motivated to quit are derived
PAU, include smoking cessation and avoidance of
from the fact that cigarette smoking is considered the
secondhand smoke. Effective strategies in those patients
largest modifiable risk factor for AAA. The use of e-
motivated to quit smoking use the 5 A’s (Ask, Advise,
cigarettes is effective in smoking cessation; however,
Assess, Assist, and Arrange) and may include dedicated
given its association with adverse vascular remodeling,
multidisciplinary programs, app-based tools, or pharma-
more evidence on its safety in patients with AAA is
cotherapy with nicotine replacement, bupropion, vareni-
1-3 needed.
cline, or all 3. Although e-cigarette use has been shown
4
to be an effective strategy in smoking cessation, the ef- 6.4.2.4. Antithrombotic Therapy in AAA
ficacy and safety of its use in patients with AAA has not
been shown.
Recommendation for Antithrombotic Therapy in AAA
1. In patients with AAA with concomitant atheroma and/or PAU, the use of low-dose aspirin may be
2b C-LD considered, unless contraindicated. 1
Synopsis study of the efficacy of antiplatelet therapy in AAA is

Atherosclerotic AAA are associated with a >20% risk of warranted.
cardiovascular events within 10 years, as they are
considered a coronary artery disease equivalent according
2
to the NCEP ATP III. To reduce risk of cardiovascular 1. Low-dose aspirin monotherapy in patients with non-
events and mortality, aspirin at 75 mg to 162 mg daily for coronary atherosclerosis is considered a treatment to
secondary prevention has been incorporated into the 2006 mitigate risk of cardiovascular events, including stroke,
updated “AHA/ACC Guidelines for Secondary Prevention death caused by coronary artery disease, and myocar-
for Patients With Coronary and Other Atherosclerotic dial infarction.3 Data are limited on aortic-specific
3
Vascular Disease.” Most AAA contain an intraluminal clinical outcomes in AAA. Use of low-dose aspirin has
thrombus (see Section 6.2, “AAA: Cause, Risk Factors, and been hypothesized to reduce growth and progression of
Screening”) made up of a complex matrix of platelets, AAA attributable to the detrimental effects of platelet
inflammatory cells, and fibrin, which contributes to activation within the intraluminal thrombus. In 1 small
growth and progression, and thus antithrombotic therapy cohort study, low-dose aspirin was associated with a
has been hypothesized to have a potential benefit in AAA. reduced AAA growth rate and need for aneurysm repair
However, clinical outcomes data are limited, and further at diameters of 4.0 cm to 4.9 cm but not for
aneurysms <4.0 cm. However, evidence from the aspirin use and the risk of AAA rupture (adjusted OR,
Danish National Registry of Patients study of 4,010 age- 0.97; 95% CI, 0.86–1.08).
and sex-matched subjects with AAA 1 showed an
increased case-fatality rate associated with preadmis-
6.4.3. Surveillance for Medical Management
sion aspirin use in ruptured AAA (66% in users versus
57% in nonusers; adjusted mortality rate ratio, 1.16; 6.4.3.1. Surveillance of Thoracic Aortic Dilation and Aneurysm
95% CI, 1.06-1.27); there was no association between
Recommendations for Surveillance of Thoracic Aortic Dilation and Aneurysm
1. In patients with a dilated thoracic aorta, a TTE is recommended at the time of diagnosis to assess aortic
1 C-LD valve anatomy, aortic valve function, and thoracic aortic diameters. 1-4
2. In patients with a dilated thoracic aorta, a CT or MRI at the time of diagnosis is reasonable to assess
2a C-LD thoracic aortic anatomy and diameters.1,3,5-7
3. In patients with a dilated thoracic aorta, follow-up imaging (with TTE, CT, or MRI, as appropriate based on
2a C-LD individual anatomy) in 6 to 12 months is reasonable to determine the rate of aortic enlargement; if stable,
surveillance imaging every 6 to 24 months (depending on aortic diameter) is reasonable.1,3,4
Synopsis segments of thoracic aorta including arch branch ves-

In patients with TAD, a detailed baseline assessment of sels.5,6 Electrocardiographic-gated techniques mini-
all the segments of thoracic aorta, aortic valve anatomy, mize motion artifact and thus allow precise
and aortic valve function is important. TTE, CT, and MRI measurement of aortic root and ascending aortic
are all commonly used for assessment of the thoracic dimensions.5,6
aorta. 3. Patients with stable aortic dimensions can be
observed longitudinally with TTE, CT, or MRI. The
frequency of surveillance imaging should be individ-
1. In patients with TAD not at surgical threshold, a detailed ualized and informed by the aneurysm cause, aortic
assessment with a TTE to evaluate aortic valve anatomy diameter, historical rate of aortic growth, how close
and aortic valve function is important for establishing a the diameter is to the surgical threshold, and the
baseline. TTE usually provides clear images of the aortic patient’s age.8,9 In general, in patients with nonge-
root and ascending aorta, is safe and reproducible, and netic and syndromic causes, the rate of aortic growth
can be used for longitudinal surveillance. In select pa- is relatively slow, so the interval for surveillance
tients with difficult echocardiographic imaging win- imaging may be increased.
dows, a TEE is an alternative for evaluating aortic valve
6.4.3.2. Surveillance of Abdominal Aortic Dilation
anatomy and aortic dimensions. 1-3
and Aneurysm
2. Cross-sectional imaging with CT or MRI has been
established as the gold standard for assessment of all
Recommendations for Surveillance of Abdominal Aortic Dilation and Aneurysm

1. In patients with an AAA of 3.0 cm to 3.9 cm, surveillance ultrasound is recommended every 3 years to
1 B-NR assess for interval change.1-8
2. In men with an AAA of 4.0 cm to 4.9 cm and in women with an AAA of 4.0 cm to 4.4 cm, surveillance
1 B-NR ultrasound is recommended annually to assess for interval change.1-8
3. In men with an AAA of ‡5.0 cm and women with an AAA of ‡4.5 cm, surveillance ultrasound is recom-
1 B-NR mended every 6 months to assess for interval change.1-8
(Continued)
4. In patients with an AAA that is inadequately defined with ultrasound, surveillance CT is recommended.
1 C-EO
In such patients, when there is a contraindication to CT or to lower cumulative radiation risk, surveillance
2a C-LD MRI is reasonable.9,10
5. In patients with an AAA that meets criteria for repair, CT is recommended for preoperative planning.
1 C-EO
Synopsis 3.9 cm, longer surveillance imaging intervals have

In patients with AAA, imaging assessment of the been shown to be safe.
abdominal aorta is important for establishing baseline 2. In patients with AAA of 4.0 cm to 4.9 cm, rates of aortic
diameter and determining the timing of surveillance im- growth are higher, so annual surveillance ultrasound is
aging. Ultrasound imaging has been the standard for recommended. Even shorter intervals are often used in
surveillance imaging of the abdominal aorta and is widely those who smoke, have diabetes, or both because of
used. CT provides superior visualization of the abdominal their increased risk of growth.
aorta and its branches and is therefore used for preoper- 3. Once the size of the AAA reaches $5.0 cm in men
ative planning. MRI is a reasonable alternative to CT in and $4.5 cm in women, the screening interval is
selected patients. Figure 20 shows a proposed general shortened to every 6 months given the potential of
algorithm for surveillance imaging of AAA, recognizing larger aneurysms to grow more rapidly and reach the
that surveillance intervals should be individualized. thresholds for intervention. CT provides superior
visualization of the abdominal aorta and its branches
and is an excellent alternative when ultrasound is
1. Multiple studies have established that ultrasound inadequate. MRA is a reasonable alternative to CT.
surveillance of AAAs helps to prevent rupture and Non-IV contrast MRI techniques have also been shown
mortality.2-7,11 The risk of rupture increases at an AAA to be useful in defining AAAs. 9,10
diameter of >5.5 cm for men and >5.0 cm for women; 4. CT is generally preferred when an AAA reaches the
accordingly, surveillance imaging should be more threshold for intervention, both to confirm aortic di-
frequent at larger AAA diameters that approach these ameters and to detail the anatomy of the aorta and its
thresholds. Conversely, at AAA diameters of 3.0 cm to branches for preoperative planning.
F I G U R E 2 0 The Frequency of Surveillance Imaging of Abdominal Aortic Aneurysms Based on Current Aortic Diameter
Color corresponds to Class of Recommendations in Table 2.

6.5. Surgical and Endovascular Management of from the aneurysmal sac. To date, the FDA has approved
Aortic Aneurysms individual stent grafts for the treatment of aneurysms
Most patients with TAA and AAA are asymptomatic, so the involving the descending thoracic, juxtarenal, and
purpose of surgical or endovascular intervention is to infrarenal aortic segments. Stent graft devices to address
reduce the risk of adverse aortic events (ie, aortic the ascending aorta, aortic arch, and thoracoabdominal
dissection, rupture, and aortic-related death). Conse- aorta are available under investigational use in the United
quently, determining the optimal timing of intervention States, currently in physician- and industry-sponsored
requires a careful anatomic assessment, followed by clinical trials. Long-term studies have shown that use of
weighing the risk of future adverse aortic events against endovascular stent grafts outside of the anatomic criteria
the risk of intervention. tested in their pivotal trials is associated with increased
The goal of open surgery is to replace the aneurysmal risk of aneurysm sac enlargement, underscoring the need
aortic segment with a prosthetic graft anastomosed to for appropriate patient selection and for long-term sur-
nonaneurysmal aortic tissues while maintaining critical veillance after endovascular repair. 1
aortic branch vessels. Endovascular repair leverages
6.5.1. Surgery for Sporadic Aneurysms of the Aortic Root and
contiguous nonaneurysmal aortic or iliac segments for
Ascending Aorta
fixation of endovascular stent grafts to exclude blood flow
Recommendations for Surgery for Sporadic Aneurysms of the Aortic Root and Ascending Aorta
1. In patients with aneurysms of the aortic root and ascending aorta who have symptoms attributable to the
1 C-LD aneurysm, surgery is indicated. 1,2
2. In asymptomatic patients with aneurysms of the aortic root or ascending aorta who have a maximum
1 B-NR diameter of ‡5.5 cm, surgery is indicated. 3-9
3. In patients with an aneurysm of the aortic root or ascending aorta of <5.5 cm, whose growth rate
1 C-LD confirmed by tomographic imaging is ‡0.3 cm/y in 2 consecutive years, or ‡0.5 cm in 1 year, surgery is
indicated.10-13
4. In asymptomatic patients with aneurysms of the aortic root or ascending aorta who have a maximum
2a B-NR diameter of ‡5.0 cm, surgery is reasonable when performed by experienced surgeons in a Multidisci-
plinary Aortic Team. 14-17
5. In patients undergoing repair or replacement of a tricuspid aortic valve who have a concomitant aneurysm
2a B-NR of the ascending aorta with a maximum diameter of $4.5 cm, ascending aortic replacement is reasonable
when performed by experienced surgeons in a Multidisciplinary Aortic Team.18-21
In patients undergoing repair or replacement of a tricuspid aortic valve who have a concomitant aneurysm
2a B-NR of the ascending aorta with a maximum diameter of ‡5.0 cm, ascending aortic replacement is reason-
able. 18-21
In patients undergoing cardiac surgery for indications other than aortic valve repair or replacement who
2b C-LD have a concomitant aneurysm of ascending aorta with a maximum diameter of ‡5.0 cm, ascending aortic
replacement may be reasonable.18
6. In patients with a height >1 standard deviation above or below the mean who have an asymptomatic
2a C-LD aneurysm of the aortic root or ascending aorta and a maximal cross-sectional aortic area/height ratio
of ‡10 cm 2/m, surgery is reasonable when performed by experienced surgeons in a Multidisciplinary
Aortic Team.14,15,22
7. In asymptomatic patients with aneurysms of the aortic root or ascending aorta who have either an ASI
2b C-LD of ‡3.08 cm/m2 or AHI of ‡3.21 cm/m, surgery may be reasonable when performed by experienced
surgeons in a Multidisciplinary Aortic Team. 23
Synopsis associated with an increased risk of rupture. 12 Because

Elective surgery for aneurysms of the aortic root and of the inherent error in measurement as well as inter-
ascending aorta is ideally performed when the risk of observer variability, 1 mm to 2 mm growth per year
adverse events—dissection, rupture, or sudden death— would be difficult to document consistently on sur-
outweighs the risks of surgery. No prospective multi- veillance imaging. Discrepancies in measurement can
center observational studies have evaluated the myriad of occur when comparing different imaging modalities or
parameters (eg, aortic diameter, length, or area, alone or even when using the same modality when comparing
indexed to height or BSA, wall stress, shear stress) pro- images obtained with and without contrast. Ideally,
posed for predicting the risk of aortic adverse events. growth rates are most accurate when assessed using
From a purely mechanical perspective, aortic dissection cardiac-gated CT or MRI with centerline measurement
or rupture can be considered a failure event, where an techniques.35 Confirmed growth of $0.5 cm in 1 year
imbalance exists between stresses on the aneurysm wall has been, and remains, an indication for surgery. 3-6
24
and the inherent strength of its tissue. Whether the Moreover, growth of even 0.3 cm in 1 year still sub-
aortic dissection is precipitated by increased wall stress or stantially exceeds the expected growth rate for aneu-
decreased wall strength, or a combination of both, is an rysms of the root and ascending aorta, so if that rate of
area of active research.25-29 Maximal aortic diameter has growth rate is sustained for 2 consecutive years,
logically been the primary criterion for elective aneurysm intervention is also recommended. 13
repair because, per LaPlace’s law, wall stress increases 4. The risk of aortic dissection or rupture correlates with
proportionally with aortic radius and inversely to thick- increasing aneurysm diameter,7,8,16 as does the rate of
30
ness. The original natural history studies examined the aortic growth. 12,36 As such, aneurysms of $5.0 cm
risk of rupture or aortic dissection versus diameter and would be expected to have a greater risk of complica-
the hinge point for dissection generated the 5.5-cm tions or rapid growth than would smaller aneurysms.
threshold that has long governed clinical practice.7,8 Indeed, in a report by Paruchuri et al, 37 relative to a
Although a significant proportion of patients with type A control aortic diameter of #3.4 cm, a diameter of 4.0
aortic dissection present with diameters <5.5 cm,31,32 this cm to 4.4 cm conferred an 89-fold increased risk of
surgical threshold still effectively reduces adverse aortic dissection, and a diameter of $4.5 cm conferred
events.17,33 a 6,300-fold increased risk (Figure 5). Consequently,
many experienced surgeons in a Multidisciplinary
Aortic Team choose to operate selectively on patients
1. Large aneurysms can compress nearby structures as with aneurysms of 5.0 cm to 5.4 cm, 17 provided the
they expand, resulting in symptoms of chest or back patient’s surgical risk is low, 38 and they have had
pain. Alternatively, pain is sometimes associated with excellent results14-16 in doing so. However, there is an
rapid aortic growth. Consequently, the appearance of ongoing prospective multicenter RCT of patients with
such symptoms raises concern for an increased risk of ascending TAAs of 5.0 cm to 5.4 cm that will compare
aneurysm rupture,1,2 and surgical repair is therefore outcomes of early elective surgery vs. medical sur-
indicated. veillance,39 the results of which could provide further
2. A maximum aortic diameter of $5.5 cm has been the guidance.
primary criterion for elective surgical repair of aneu- 5. For patients undergoing aortic valve surgery with
rysms of the aortic root or ascending thoracic aorta, 4,6 concomitant ascending aortic aneurysm of $4.5 cm,
based on natural history studies that examined diam- guidelines have previously recommended simulta-
eter (without centerline analysis) at the time of adverse neous aortic replacement in those with BAV. On the
event and an assumed operative mortality of <5%.4,7-9 other hand, in patients who have undergone valve
The mortality rate for elective surgery is low, whereas surgery without concomitant aortic aneurysm surgery,
the risk of adverse events is high when such surgery is whether for an underlying bicuspid or tricuspid aortic
recommended but not performed because of patient valve, the associated aneurysms have been shown to
noncompliance or comorbidities.33 The same 5.5-cm grow slowly and have low rates of aortic complications
diameter threshold applies regardless of whether pa- over time. Still, data have also shown the safety of
tients have tricuspid or BAVs. 5 performing concomitant aneurysm repair at a diameter
3. One meta-analysis and limited observational studies of $4.5 cm by experienced surgeons working in a
have found ascending aortic aneurysm growth to be Multidisciplinary Aortic Team. 16,18-21,40-43 Neverthe-
slower than previously reported, and frequently <0.5 less, until there are better predictors for aortic com-
mm/y, in patients with a tricuspid aortic valve and plications, in general it is reasonable in patients
without a genetic aortic disorder.11,12,17,34 The meta- undergoing aortic valve repair or replacement to offer
analysis suggested that rapid aneurysm growth is concomitant aneurysm surgery for those with
aneurysms of $5.0 cm, because of the faster rate of prospectively validated. A cross-sectional aortic area to
growth and higher risk of aortic dissection. Aortic root patient height ratio of $10 cm 2/m was found to corre-
replacement should be individualized based on the late with increased mortality among unoperated pa-
type of aortic valve surgery (ie, valve repair with or tients with root or ascending aortic aneurysms and
without valve-sparing root versus valve replacement, either a tricuspid15 or BAV.14 The use of the cross-
mechanical versus bioprosthetic root replacement), sectional aortic area to height ratio is most appro-
patient condition, patient age, and comorbidities. In priate in patients whose height is >1 standard devia-
those undergoing cardiac surgery for indications other tion above or below the mean.
than aortic valve repair, concomitant prophylactic 7. A single-center large database of TAA has grown
aortic replacement at a diameter of 5.0 cm may be considerably and was reevaluated with indexing of
reasonable, because it would provide a margin of aortic diameter to BSA (ASI) or height (AHI), to improve
safety against future aortic dissection, particularly the prediction of adverse aortic events. 23 Height was
because cardiac surgery itself becomes an additional preferred because the variable nature of weight and the
risk factor for subsequent aortic dissection. underlying genetic contribution to height. Recommen-
6. Data from the IRAD showed that w60% of patients with dations for prophylactic repair at aortic diameters
acute type A aortic dissection had maximal aortic di- of <5.5 cm have been proposed but not systematically
ameters of <5.5 cm32 at presentation, a finding that has tested in large-scale multicenter trials. Experienced
been corroborated by others. 31,44 Conversely, most surgeons in a Multidisciplinary Aortic Team 38 may
patients with aneurysms <5.5 cm who are managed consider the use of such ratios when determining the
medically do not suffer aortic dissection or rupture. optimal timing of intervention. This may be particular
Therefore, absolute aortic diameter is far from an ideal useful for female patients, but more studies are required
predictor of risk. Parameters proposed to improve risk to further evaluate surgical thresholds in women with
prediction include the ratio of aortic diameter to either aneurysms of the aortic root or ascending aorta.
patient height or BSA, 23 the ratio of aortic area to
height,14,15 the ascending aortic length (centerline,
6.5.1.1. Surgical Approach for Patients With Sporadic
from annulus to innominate artery takeoff), 14,15,45-47
Aneurysms of the Aortic Root and Ascending Aorta
aortic stiffness, and peak aortic wall stress.25,48-50 All
Meeting Criteria for Surgery
are retrospectively promising, but none has been
Recommendations for Surgical Approach for Patients With Sporadic Aneurysms of the Aortic Root and
Ascending Aorta Meeting Criteria for Surgery
Referenced studies that support the recommendations are summarized in the Online Data Supplement
1. In patients with an aneurysm isolated to the ascending aorta who meet criteria for surgery, aneurysm
1 B-NR resection and replacement with an interposition graft should be performed.1,2
2. In patients undergoing aortic valve repair or replacement with a concomitant ascending aortic aneurysm,
1 B-NR a separate aortic valve intervention and ascending aortic graft is recommended. 3-6
3. In patients undergoing aortic root replacement with an aortic valve that is unsuitable for sparing or
1 B-NR repair, a mechanical or biological valved conduit aortic root replacement is indicated. 1,2,7,8
4. In patients undergoing aortic root replacement, valve-sparing aortic root replacement is reasonable if the
2a B-NR aortic valve is suitable for sparing or repair and when performed by experienced surgeons in a Multi-
disciplinary Aortic Team.9-21
Synopsis aneurysm repair is acceptable, because data suggest

The goal of prophylactic repair of aneurysms of the the aortic root dilates at a slower rate than does the
aortic root and ascending aorta is to prevent life- ascending aorta, and studies of root-sparing surgery
threatening complications from acute aortic events such have shown no increase in long-term adverse aortic
as aortic dissection, aortic rupture, or sudden death. This events. 3-6,25
goal is best achieved when the risk of future adverse 3. Surgical approaches to replace the aortic root should be
aortic events is greater than the expected surgical mor- guided by the aortic valve anatomy. If the aortic valve
tality (considering both the surgeon’s and institutional is unsuitable for sparing or repair (eg, large fenestra-
experience). The STS database has clearly shown that tions, calcification), a mechanical- or biological-valved
proximal thoracic aortic surgery has a lower operative conduit aortic root replacement should be performed
mortality when performed electively rather than emer- because, when elective, this procedure has an opera-
gently (2.2% versus 17.2%, respectively).1 tive mortality rate of 2.2% in the United States based on
the STS database.2,26 Single-institution series from
centers with Multidisciplinary Aortic Teams have also
1. Proximal thoracic aortic operations in the United shown excellent results both with and without
States, including ascending thoracic aortic replace- concomitant hemiarch replacement. 7 Long-term out-
ment and aortic root replacement, have an overall comes are similar with mechanical- versus biological-
elective mortality rate of 2.2%. Consequently, patients valved conduit aortic root replacements, even in
who meet criteria for aneurysm repair and have low patients <70 years old.8
operative risk can undergo prophylactic resection and 4. In younger patients with an aortic valve that is
graft placement with low operative mortality risk. 1 amenable to sparing or repair, elective valve- sparing
Similar results were obtained when examining the aortic root replacement has been performed safely by
Nationwide Inpatient Sample in which operative mor- experienced surgeons in a Multidisciplinary Aortic
tality rate for proximal thoracic aortic surgery was Team.9-11,21 In patients with aortic root aneurysms
2
2.5%. without an underlying genetic disorder, valve-sparing
2. Single-institution studies have shown that the addition aortic root replacement has been performed by either
of ascending TAA repair to AVR does not increase the reimplantation or remodeling technique with
operative mortality in experienced aortic centers. 22-24 comparable survival and valve durability.12
However, such results may not be reproducible at
low-volume centers that have a higher operative mor-
6.5.2. Aortic Arch Aneurysms
tality rate for isolated proximal thoracic aortic sur-
gery.1,2 Root-sparing AVR with concomitant ascending
Recommendations for Aortic Arch Aneurysms

1. In patients with an aortic arch aneurysm who have symptoms attributable to the aneurysm and are at low
1 C-EO or intermediate operative risk, open surgical replacement is recommended.
2. In patients with an isolated aortic arch aneurysm who are asymptomatic and have a low operative risk,
2a B-NR open surgical replacement at an arch diameter of ‡5.5 cm is reasonable.1-3
3. In patients undergoing open surgical repair of an ascending aortic aneurysm, if the aneurysmal disease
2a C-LD extends into the proximal aortic arch, it is reasonable to extend the repair with a hemiarch
replacement.4,5
4. In patients undergoing open surgical repair of an aortic arch aneurysm, if the aneurysmal disease extends
2b C-LD into the proximal descending thoracic aorta, an elephant trunk procedure may be considered.6,7
5. In patients with an aortic arch aneurysm who are asymptomatic but meet criteria for intervention, but
2b C-EO have a high risk from open surgical repair, a hybrid or endovascular approach may be reasonable.
Synopsis neuroprotective strategies. Modified approaches have

Aortic arch aneurysms are the least common of the been described that eliminate the need for open repair
TAA, because <10% of aneurysms involve the arch only; whereby the ascending aorta is replaced first with a
in most cases, arch aneurysms are associated with adja- trifurcated side-branch for debranching of the arch in a
cent pathology. 1 Previous aortic dissection is the most sequential manner to a level that is accessible for
common cause of arch aneurysms; in a large meta- clamping; however, no studies have yet shown the
analysis, only 28.3% of patients undergoing intervention benefit of such an approach. Although it does add to
on the arch had de novo aneurysmal disease, with the the cardiopulmonary bypass time and blood loss, 4,5 the
remainder resulting from acute or chronic aortic dissec- addition of a hemiarch has been shown not to increase
tion. The risk of dissection or rupture, as related to aortic the risk of the procedure. However, this noninferiority
diameter, is presumed to be similar in the arch as in other is lost when the proximal arch is disease free, with
thoracic locations, although no large reports consider arch exception of an underlying aortopathy in which the
dimensions alone. Additionally, because of the proximity normal-sized arch will predictively enlarge or dissect at
of the aortic arch to other thoracic structures, dilation a later time; in this setting, a hemiarch is justified.16,17
may result in symptoms before the diameter reaches a 4. The elephant trunk procedure, as originally described,
threshold typically considered for intervention. Inter- extends the aortic arch graft into the proximal
vention to treat arch aneurysms carries an increased risk descending aorta, thereby facilitating the subsequent
given the need to manage the great vessels and protect repair of diseased descending thoracic aorta (by either
the brain. Various techniques have been developed, open repair or TEVAR).18 Either a traditional elephant
including the use of hypothermic circulatory arrest and trunk (an extension graft anastomosed to the distal end
specialized branched grafts to aid in reconstruction. of an aortic arch graft at the time of arch repair that
Endovascular techniques also continue to evolve. projects into the proximal descending aorta with a free
distal end) or a frozen elephant trunk (a combined open
aortic arch graft with an extension endovascular stent-
1. Because of the juxtaposition of the aortic arch to other graft extending into the descending thoracic aorta to
vascular structures, nerves, trachea, and the esoph- treat extensive TAD involving both arch and descending
agus, symptoms may develop because of the mass ef- segments via a median sternotomy) can be used. 6,19
fect from encroachment on adjoining structures. With adjunctive procedures (ie, debranching), the
Ortner’s syndrome is unilateral hoarseness secondary distal anastomosis can be moved more proximally into
to inflammation or stretching of the left recurrent aortic zones 2 or 3 (Figure 3), while still proceeding with
laryngeal nerve.8 Dysphagia aortica can be caused by an elephant trunk and with the potential of decreasing
extrinsic compression of the esophagus by either fusi- morbidity, but data are limited on the benefits of mov-
form or saccular aneurysms of the arch. Likewise, ing the anastomotic site. A qualifying factor for
extrinsic compression of the trachea may result in considering open versus frozen elephant trunk is
dyspnea, and compression of the innominate vein or whether the primary distal seal will be achieved with
superior vena cava may cause superior vena cava the frozen elephant trunk. In the absence of a distal
syndrome. Nonspecific symptoms include chest pain or seal, the conventional approach would provide the
pressure, fatigue, and neck, jaw, or back pain. same considerations for the second-stage procedure.
2. Open replacement of the aortic arch requires the use of 5. Various hybrid and endovascular techniques have been
cardiopulmonary bypass, hypothermia, and other ad- developed to address the aneurysmal arch in the
juncts for neurologic and systemic protection. Various setting of a high-risk patient, including open extra-
randomized and nonrandomized trials have compared anatomical bypasses (eg, left carotid-to-left subcla-
different cannulation strategies (ie, axillary, femoral, vian artery bypass) and endovascular approaches. The
innominate), 9-12 levels of hypothermia, and variations Next-gen Fenestrated TEVAR trial showed the feasi-
in cerebral perfusion (antegrade, select antegrade, bility of proximal landing zone coverage, with most
retrograde),13-15 with no one technique dominating or endografts being placed in zones 0 or 1 (Figure 3),
being shown conclusively to be superior to another. although a landing zone of <15 mm was associated with
3. When proximal aneurysmal aortic disease extends to an increased risk of a type I endoleak (Figure 11).20 The
the level of the innominate artery or further into the midterm follow-up showed 5-year survival of 71% with
arch, but not necessarily the whole arch, a hemiarch an aneurysm-related event-free survival of 77%. The
procedure may be able to effectively address the distal most frequent reason for reoperation was type Ia
pathology. Open distal anastomosis will require the endoleak (5%).21 Nonrandomized comparisons of open
same adjuncts and approaches used in open arch versus hybrid endovascular approaches have not
replacement (as described previously), including shown significant differences in outcomes.22-24
Complete endovascular approaches have been 6.5.3. Descending TAA

described 25,26 and may be considered by those with 6.5.3.1. Size Thresholds for Repair of Descending TAA
endovascular experience who have access to the
appropriate devices, investigational devices, or both.
Recommendations for Size Thresholds for Repair of Descending TAA

1. In patients with intact descending TAA, repair is recommended when the diameter is ‡5.5 cm. 1,2
1 B-NR
2. In patients with intact descending TAA and risk factors for rupture (Table 17), repair may be considered at
2b B-NR a diameter of <5.5 cm. 2-6
3. In patients at increased risk for perioperative morbidity and mortality (Table 18), it may be reasonable to
2b B-NR increase the size threshold for surgery accordingly.7

The current aortic size threshold for repair of
1. There is an increased incidence of aortic-related events
descending TAA is primarily based on single-center series
such as rupture or dissection with aortic diameters >6
where patients have been observed with surveillance im-
cm, justifying intervention when the diameter is $5.5
aging and clinical follow-up to determine the incidence of
cm in size.1,2
aortic-related events and deaths. Such series indicate that
2. High-risk features of rupture have been previously
a descending aortic diameter of >6 cm is associated with
identified, supporting repair at a smaller diameter
an increased risk of adverse aortic events and mortality,1,2
threshold when these criteria are met. Features
as shown in Table 16. Moreover, certain patient and
including rapid aortic growth ($0.5 cm/y), 3 symptom-
aneurysm features are associated with an increased risk
atic aneurysms,4 underlying connective tissue disorder
for aortic dissection or rupture, as shown in Table 17,
or HTAD, 2 saccular aneurysm morphology,5 female
which may prompt consideration of earlier surgery.
sex,2 and infected aneurysm 6 have all been associated
Conversely, some patients are at increased risk of peri-
with a higher tendency for rupture.
operative morbidity or mortality, in which case the size
3. In patients being considered for open or endovascular
threshold for aortic repair might be increased. Specifically,
repair, high-risk clinical features (Table 18) have been
if the patient does not have ideal anatomy for endovas-
identified that portend poor outcomes after repair.
cular repair, or has otherwise increased risk for contem-
For open surgical repair, advanced age,8 preoperative
plated open repair, close monitoring until a higher surgical
renal insufficiency of stage 3 or greater, chronic
threshold is reached would be justified. Advanced age,8
obstructive pulmonary disease and forced expiratory
preoperative renal insufficiency or hemodialysis, chronic
volume in 1 second #50% predicted, and previous
obstructive pulmonary disease, and previous stroke are
stroke9 have all been associated with increased risk of
harbingers of adverse outcomes or perioperative mortality
death, perioperative morbidity, or both. For endo-
after open repair (Table 18). 9 Markers of frailty, pulmonary
vascular repair of descending TAA, frailty indicators,
disease, thoracoabdominal extent, need for iliac access,
pulmonary disease, as well as procedural complexity
and zone 1/2 deployment were associated with major
are predictive of poor outcomes after TEVAR.7 When
adverse events after TEVAR (Table 18). 7 A nuanced
contemplating either approach, special attention to
approach and detailed discussion with the patient can
these risk factors will allow appropriate consideration
help guide the most reasonable treatment plan, weighing
of the risks to benefits in deciding on the merits of
the risks of the operation against the risks of continued
intervention.
surveillance.
Adverse Aortic Events at 1 Year, Based on TABLE 17 Risk Factors for Aortic Rupture Among Patients
TABLE 16 Baseline Aortic Diameter, Among Patients With With Descending TAA
Descending TAA
High-Risk Features for Rupture
Aortic Diameter Definite Aortic Event* Probable Aortic Event† Aneurysm growth of $0.5 cm/y3
(cm) (%) (%)
Symptomatic aneurysm4
5.0 5.5 8.0
Marfan, Loeys-Dietz, or vascular Ehlers-Danlos syndrome, or HTAD
5.5 7.2 11.2
(see Section 6.1.2, “Genetic Aortopathies”)2
6.0 9.3 15.6
Saccular aneurysm5
7.0 15.4 28.1
Female sex2
Based on data from Kim JB, et al.1 Infectious aneurysm6
*Definite aortic event includes aortic dissection or rupture confirmed with imaging or
intraoperative findings. HTAD indicates heritable thoracic aortic disease; and TAA, thoracic aortic aneurysm.
†Probable aortic event includes definite aortic events as well as sudden unexplained
death.
TAA indicates thoracic aortic aneurysm.
TABLE 18 Patient Characteristics Associated With Increased Perioperative Morbidity and Mortality After Open and
Endovascular Repair of Descending TAA
Open Surgical Repair Endovascular Repair

Advanced age8 Functional dependence
65-74 y (OR, 1.8; 95% CI, 1.4-2.4; P<0.001)
$75 y (OR, 2.6; 95% CI, 2.0-3.5; P<0.001)
Preoperative renal insufficiency (stage 3 or greater CKD) or hemodialysis Thoracoabdominal aortic aneurysm extent
COPD and FEV1 #50% predicted Pulmonary disease
Previous stroke9 Need for iliac access
Zone 1/2 landing for thoracic stent graft7
CKD indicates chronic kidney disease; COPD, chronic obstructive pulmonary disease; FEV1, forced expiratory volume in 1 second; and TAA, thoracic aortic aneurysm.
6.5.3.2. Endovascular Versus Open Repair of Descending TAA
Recommendations for Endovascular Versus Open Repair of Descending TAA

1. In patients without Marfan syndrome, Loeys-Dietz syndrome, or vascular Ehlers-Danlos syndrome, who
1 B-NR have a descending TAA that meets criteria for intervention and anatomy suitable for endovascular repair,
TEVAR is recommended over open surgery.1-4
2. In patients with a descending TAA that meets criteria for repair with TEVAR, who have smaller or diseased
1 B-NR access vessels, considerations for alternative vascular access are recommended. 5
3. In patients with a descending TAA that meets criteria for intervention, who have anatomy unsuitable for
2a B-NR endovascular repair, and who are without significant comorbidities and have a life expectancy of at least
10 years, open surgical repair is reasonable. 6-9
Synopsis lower (41% versus 84%, respectively; P<0.01) for

Although no RCTs comparing TEVAR with open repair TEVAR; at 1 year, aneurysm-related mortality rate was
of descending TAA exist, the pivotal device trials1,3,10 lower for TEVAR than for open repair (3.1% versus
have shown a reduced perioperative morbidity, 11.6%, respectively; P<0.002). However, in a registry
increased clinical utility, and reduced follow-up aneu- study using Medicare claims data,4 although short-
rysm-related mortality compared with open surgical term outcomes were similarly better with TEVAR
repair. However, reintervention after TEVAR is substan- compared with open repair, that survival advantage
tial. 11 In addition, although clinical device trials showed was no longer present at 1 year and, at 5 years, survival
improved perioperative and long-term outcomes with was significantly worse for TEVAR versus open repair
TEVAR, Medicare claims data show that the perioperative at 79% versus 89%, respectively (P<0.0001). Overall,
advantage was lost within the first year after intact the data show that TEVAR is beneficial in the short- to
aneurysm repair, with a 5-year survival that was signifi- intermediate-term in patients with appropriate anat-
cantly worse after TEVAR versus open repair, at 79% omy for endovascular repair, but the advantage is not
versus 89%, respectively (P<0.0001). 4 Further study sustained over time.
should be dedicated to understanding why the benefit 2. Because of the relatively large delivery systems for
from endovascular repair decays over time. In patients thoracic endografting, iliac artery graft conduits may
with connective tissue disorders or HTAD, or those with a be required to ensure safe delivery of the endograft
longer life expectancy, open surgical repair is reasonable. into the aorta. In the clinical device trials, access of
Open surgical repair of descending TAA reflects a volume- vessels other than the femoral artery was required in
outcomes relationship: Although large institutional series 9.4% to 21.1% of patients because of small or diseased
have shown good outcomes with open repair,6-9 these access vessels.1-3 In a multicenter cohort study from
12
results are not replicable at lower volume centers. The the GREAT (Global Registry for Endovascular Aortic
decision to proceed with endovascular versus open repair Treatment) registry,5 the overall access complication
balances the need for appropriate anatomy and access, as rate was 2.8%, and women had a higher rate of access
well as a higher reintervention rate for TEVAR versus the complications than men (4.7% versus 1.8%, respec-
higher perioperative risk associated with more definitive tively; P¼0.013), with a higher rate of the need for iliac
open surgical repair. and aortic access or surgical conduit, as well as access
vessel thrombosis irrespective of the clinical setting,
type of aortic disease, and device sizing.
1. TEVAR is associated with a reduced perioperative 3. Open descending thoracic aortic repair can be per-
morbidity, reduced hospital length of stay, and better formed with low morbidity and mortality rates in high-
freedom from aneurysm-related mortality compared to volume centers.6-8,14 In a multicenter retrospective
open surgical repair, based on clinical device trial study using the MEDPAR (Medicare Provider Analysis
data.1,3,10 In the study by Makaroun et al in 2008, 13 140 and Review) data,15 the overall mortality rate after
patients with fusiform aneurysms were treated with open surgical repair of descending TAA decreased in
TEVAR and compared with 94 open surgical controls. high-volume versus low-volume centers (11% versus
At 5 years, there was a decreased aneurysm-related 15%; P<0.01). In addition, data using Medicare claims
mortality (2.8% versus 11.7%, respectively, P¼0.008), show that the benefit of TEVAR is no longer present 1
a reduced major adverse event rate (57.9% versus year after endovascular therapy, with a significantly
78.7%, respectively, P¼0.01), and decreased major worse 5-year survival compared with open repair (79%
aneurysm-related reintervention (3.6% versus 2.1%, versus 89%; P<0.0001). 4 In a recent retrospective,
respectively) in TEVAR versus open repair. In the study single-center study in which propensity score matching
by Matsumura et al 10 , survival was noninferior for analysis was used to compare the outcomes of open
TEVAR (98.1%) versus open surgery (94.3%) at 30 days, and endovascular descending and TAAA repair in 278
but the severe morbidity composite index, a marker for pairs of patients,16 open repair resulted in better 10-
postoperative complications, was lower for TEVAR year survival than endovascular repair (52% versus
(0.20.7 versus 0.71.2, respectively; P<0.01). In the 33%; P<0.0001). Because of the lack of available long-
study by Fairman et al,11 195 TEVAR patients were term data on aortic-specific mortality rate in young
compared with 189 open surgical controls, and the 30- patients after TEVAR, in patients deemed to have a life-
day mortality rate was lower (2% versus 8%, respec- expectancy of $10 years, open surgical repair is
tively; P<0.01) and the major adverse event rate was reasonable.
6.5.3.3. Left Subclavian Artery Management
Recommendations for Left Subclavian Artery Management

1. In patients with descending TAA who undergo TEVAR with planned left subclavian artery coverage,
1 B-NR revascularization of the left subclavian artery before TEVAR is recommended to prevent spinal cord injury
(SCI) 1,2 and potentially to reduce stroke risk2 and prevent other ischemic complications.
2. In patients with descending TAA who have undergone TEVAR with left subclavian coverage and develop
2b C-LD SCI that is unresponsive to an increase in BP or a cerebrospinal fluid drain, left subclavian artery
revascularization may be considered. 3
Synopsis coverage. Preoperative left subclavian revasculariza-

Left subclavian artery coverage is required in up to tion has been shown to decrease the rates of
40% of cases of TEVAR of descending TAAs. 4 SCI and stroke2,7,8 and SCI.1,2 Vertebrobasilar insufficiency
stroke remain devastating complications associated with and left arm ischemia can also occur without left
TEVAR. Addressing these modifiable risk factors would subclavian artery revascularization. 9,10 Patients with
allow for better outcomes after this less invasive treat- a patent left internal mammary artery to left anterior
ment strategy. In addition, special considerations include descending artery coronary artery bypass graft, or
the prevention of vertebrobasilar insufficiency (particu- who are otherwise reliant on inflow from the left
larly among those with a dominant left vertebral artery), subclavian artery (eg, for dialysis access), should
preservation of any preexisting left internal mammary undergo left subclavian revascularization to preserve
artery coronary bypass graft, as well as left upper ex- flow.9
tremity dialysis access or other left upper extremity- 2. Patients undergoing TEVAR with left subclavian
based graft. Currently, pivotal as well as feasibility trials coverage may not be hemodynamically stable enough
are ongoing for branched endografts intending to pre- to undergo preemptive revascularization of the left
serve flow to the left subclavian artery. Longer-term subclavian artery. If such patients go on to develop SCI
follow-up of this technology is needed, but initial re- after TEVAR, there have been case reports of SCI
sults are promising. 5,6 reversal with secondary revascularization of the left
subclavian artery. 3
1. Up to 40% of patients undergoing TEVAR for thoracic

6.5.3.4. Celiac Artery Management
aneurysm repair require left subclavian artery
Recommendation for Celiac Artery Management

References that support the recommendation are included in the Online Data Supplement.
1. In patients with descending TAA undergoing TEVAR in whom celiac artery coverage is being considered,
2a B-NR it is reasonable to first confirm adequate collateralization. 1
Synopsis incidence of postoperative visceral ischemia. However,

Celiac artery coverage is estimated to be necessary in despite the preoperative evaluation with CTA, angiog-
15% of patients undergoing TEVAR for descending TAA raphy, or both to confirm adequate collateralization be-
repair.2 The safety and use of this practice has previously tween the celiac and superior mesenteric artery (SMA), a
been shown with single-institution series citing low small percentage of patients still die from visceral
ischemia. In addition, late distal migration of the collateralization on CTA, angiography, or both, a small
endograft can encroach on the SMA, creating SMA ste- percentage of patients go on to develop postoperative
nosis and compromising flow through the SMA and celiac- visceral ischemia. Although the risk of visceral
based collaterals. ischemia after celiac artery coverage with TEVAR is
relatively low, there remains a finite risk (3.2% in
largest clinical series) 3 for visceral ischemic complica-
1. Migration of the endograft distally over time can cause tions, which can lead to death.
stenosis of the SMA and decrease flow to the SMA and
celiac artery-based collaterals. In patients undergoing 6.5.3.5. Ruptured Descending TAA
TEVAR with celiac artery coverage who have adequate
Recommendations for Ruptured Descending TAA

1. In patients with ruptured descending TAA who are anatomic candidates for endovascular repair, TEVAR is
1 B-NR recommended over open repair because of decreased perioperative death and morbidity.1-5
2. In patients with ruptured descending TAA undergoing TEVAR, intentional coverage of the left subclavian
2b B-NR artery, celiac artery, or both may be considered to increase the landing zone for endovascular repair. 5-7
Synopsis aneurysm-related mortality at 4 years. 1 Similarly, a

Ruptured TAA carry a high mortality rate. Single-center meta-analysis showed that TEVAR was associated with
data, meta-analyses, and clinical trials have all shown the a lower perioperative mortality and myocardial infarc-
lower rates of perioperative death and complications tion rate compared with open repair.1 A multicenter,
associated with endovascular versus open surgical prospective clinical trial for aortic catastrophes—
repair.1-5 However, the survival advantage shown in including aortic rupture—showed that TEVAR was su-
Medicare-based claims data disappears after 1.5 years, 4 perior with regard to the composite endpoint of mor-
and single-institution series 1,3 reflect the frequent need tality and paraplegia, compared with open repair. 5
for reintervention over time. Furthermore, a meta-anal- Although the perioperative benefit of endovascular
ysis 2 showed that aneurysm-related survival was repair of ruptured TAA was again corroborated in a
decreased in the TEVAR group over time, underscoring the Medicare-claims dataset, the survival advantage with
importance of continued surveillance in this high-risk TEVAR disappeared after 1.5 years.4
population. 2. When ruptured descending TAA present, coverage of
the left subclavian artery, celiac artery, or both may be
necessary to gain the necessary 2 cm of seal zone for
1. For repair of ruptured descending TAA, TEVAR is successful endovascular repair. Left subclavian artery
associated with decreased perioperative morbidity and and celiac artery coverage during thoracic aortic
mortality compared with open repair. In 1 retrospective rupture has been associated with reasonable technical
multi-institution study, TEVAR had a lower composite success and outcomes in single-institution series6 and 1
rate of death, stroke, and permanent paraplegia clinical trial 5 in patients with acute rupture or compli-
compared with open surgery and a trend toward lower cated dissection of the descending thoracic aorta.
6.5.3.6. Access Issues for TEVAR in Descending TAA
Recommendations for Access Issues for TEVAR in Descending TAA

1. In patients with descending TAA undergoing TEVAR, review of preoperative CTA of the iliofemoral vessels
1 B-NR should be performed to evaluate access. 1,2
2. In patients with descending TAA undergoing TEVAR, if iliac access is marginal or inadequate to prevent
1 B-NR access-related complications, the use of alternative conduits is recommended. 1,2
3. In patients with descending TAA undergoing TEVAR who have suitable anatomy, total percutaneous
2a B-NR femoral access is a reasonable alternative to open surgical cutdown to avoid access-related
complications.3-5
Synopsis 2. Alternative access was required in up to 21.1% of pa-

Iliac artery access for stent-graft delivery systems is tients undergoing TEVAR in the clinical device trials. 8
marginal in up to 21% of cases in which TEVAR is per- Women have a higher incidence of smaller diameter
formed for descending TAA. 1 Careful review of the CTA of external iliac arteries compared with men. 1,2 Direct
the iliofemoral system is required to ensure that marginal aortic or iliac artery exposure, iliac conduits, or endo-
or inadequate access is noted and properly managed. vascular techniques may be used to facilitate safe de-
Marginal access can be successfully circumvented using livery of endografts during TEVAR. 1,2 Preoperative case
surgical bypass, direct aortic or iliac exposure, or endo- planning will enable safe delivery of endografts
vascular techniques to treat vessel stenosis. Percutaneous without vascular complications.
access was used successfully for endovascular abdominal 3. Percutaneous access for delivery of TEVAR has been
aortic repair before it was applied to larger sheath de- performed safely and with a high degree of success,
vices. This technology has also been applied to TEVAR as shown in single-institution 4,5 as well as multi-
with a similarly high degree of success and reduced hos- institution registries. 3 Technical success ranged
pital length of stay. from 94.4% to 98.9%, and percutaneous access was
associated with fewer complications and a shorter
length of stay compared with those with surgical
1. Thoracic endovascular stent grafts are housed in large cutdown.
delivery systems, thus thorough review of the
iliofemoral system is required to avoid access compli- 6.5.4. Thoracoabdominal Aortic Aneurysms
cations. In the clinical device trials, alternative access 6.5.4.1. Size Thresholds for Open Surgical Repair of TAAA
was required in 9.4% to 21.1% of patients because of
small or diseased access vessels.6-8
Recommendations for Size Thresholds for Open Surgical Repair of TAAA

1. In patients with intact degenerative TAAA, repair is recommended when the diameter is ‡6.0 cm.1-3
1 B-NR
2. In patients with intact degenerative TAAA, repair is reasonable when the diameter is ‡5.5 cm and the
2a B-NR repair is performed by experienced surgeons in a Multidisciplinary Aortic Team.1-3
3. In patients with intact degenerative TAAA who have features associated with an increased risk of rupture
2a B-NR (Table 19), repair is reasonable when the diameter is <5.5 cm.4
chronic obstructive pulmonary disease, advanced age,

TABLE 19 Features Associated With an Increased Risk of TAAA
preoperative renal dysfunction, preoperative left ven-
Rupture
tricular dysfunction), it may be appropriate to continue
Rapid growth (confirmed increase in diameter of $0.5 cm/y)
to observe patients with TAAA diameters >6.0 cm or to
Symptomatic aneurysm
refer them for endovascular repair.
Significant change in aneurysm appearance
2. In centers with a Multidisciplinary Aortic Team,
Saccular aneurysm or presence of penetrating atherosclerotic ulcers
despite the presence of comorbid conditions, excellent
TAAA indicates thoracoabdominal aortic aneurysm. outcomes can be achieved with meticulous periopera-
Synopsis tive preparation and care as well as technically sound
The data supporting aortic diameter thresholds for surgery. On multivariable analysis, patients undergo-
either open or endovascular repair of TAAA are similar to ing TAAA repair with a left ventricular ejection
that presented for repair of descending TAA (see Section fraction <40% were not more prone to operative death
6.5.3, “Descending Thoracic Aortic Aneurysms”). All are (OR, 0.28; 95% CI, 0.02–4.14; P¼0.58) or long-term
single-institution series with longitudinal follow-up via death (OR, 0.55; 95% CI, 0.17–1.80; P¼0.23) than those
surveillance imaging and detection of aortic-related with higher ejection fractions. 5 Similarly, carefully
events and death. Intervention at diameters of <6.0 cm selected octogenarians undergoing open TAAA repair
would reduce aortic-related events and death. There are had a similar operative mortality rate as those <80
also conditions in which intervention may be justified at years of age (5.2% versus 5.7%; P¼0.852). 6
smaller diameters (eg, rapid growth, symptoms, pene- 3. Certain clinical factors associated with an increased
trating ulcers, mycotic aneurysms, connective tissue dis- risk of TAAA rupture may prompt consideration of
orders). Concerns for operative death in the setting of open or endovascular intervention at a diameter below
comorbid conditions is certainly justified. However, in the standard surgical thresholds. In patients with intact
centers with a Multidisciplinary Aortic Team, excellent TAAA who are being observed with surveillance imag-
outcomes can be obtained despite the presence of such ing, confirmed rapid aneurysm growth ($0.5 cm/y)
conditions, and fatal aortic events may thus be avoided. would suggest the need for intervention regardless of
absolute diameter. 4 Symptoms consistent with an
Recommendation-Specific Supportive Text enlarging TAAA that are not attributable to alternative
1. Aortic event rates begin to rise significantly, and 5-year pathology portend potential rupture and also suggest
survival begins to fall when TAAA diameters are >6.0 the need for surgery.7 Patients with symptoms sec-
cm. At this diameter, the risk of an adverse aortic event ondary to either PAU or saccular aneurysms are also at
1 3
ranges from 9.3% to 19%, which is 2 to 4 times the a higher risk for rupture and should be considered for
median operative mortality rate for open TAAA repair. intervention regardless of absolute diameter.8
In patients with multiple comorbidities known to sub- 6.5.4.2. Open Versus Endovascular Repair of TAAA
stantially increase the risk of open TAAA repair (eg,
Recommendations for Open Versus Endovascular Repair of TAAA

Ruptured TAAA
1. In patients with ruptured TAAA requiring intervention, open repair is recommended. 1-5
1 B-NR
2. In patients with ruptured TAAA requiring intervention, provided that the patient is hemodynamically
2b C-LD stable, endovascular repair may be reasonable in centers with endovascular expertise and access to
appropriate endovascular stent grafts. 6
Intact TAAA
3. In patients with Marfan syndrome, Loeys-Dietz syndrome, or vascular Ehlers-Danlos syndrome and intact
1 C-LD TAAA requiring intervention, open repair is recommended over endovascular repair. 7-9
4. In patients with intact degenerative TAAA and suitable anatomy, endovascular repair with fenestrated
2b B-NR stent grafts, branched stent grafts, or both may be considered in centers with endovascular expertise and
access to appropriate endovascular stent grafts.10-13
Synopsis endovascular approach is hampered by patient insta-

There are no RCTs comparing early or late outcomes for bility and the need for customized grafts (which may
open versus endovascular repair for TAAA. As of take several weeks to manufacture). In addition, most
November 2022, there are no FDA-approved devices for of the reported series of endovascular repair of
endovascular TAAA repair. Most of the endovascular ruptured TAAA are small; larger series with longer-
procedures currently performed are done so with term follow-up will be necessary to delineate the role
customized fenestrated or branched endografts on for endovascular repair in the setting of aortic rupture.
investigational device exemption- or industry-sponsored 2. Endovascular repair requires sequential steps for suc-
trials. Although the number of endovascular repairs per- cessful stenting of side branches without the ability to
formed has been steadily increasing, follow-up remains achieve rapid control of hemorrhage. Therefore, the
limited, and open repair therefore remains the preferred role of off-the-shelf branched repair has been limited
therapy for patients with TAAA who require intervention. in patients with ruptured aneurysms and hemody-
The results for open repair are excellent in centers with a namic instability. However, in higher-risk patients who
Multidisciplinary Aortic Team. In the largest series pub- present with symptomatic or contained ruptured an-
lished to date, the operative mortality rate in 3,309 pa- eurysms, are hemodynamically stable, and have suit-
tients undergoing open TAAA repair was 7.5%, including able anatomy, endovascular repair with an off-the-
>1,000 patients undergoing repair of an extent II aneu- shelf or modified device may be considered. Kolbel
rysm, with a low risk of aortic-related reintervention. et al 14 reported a mortality rate of 15% for symptomatic
Other high-volume centers have reported similar out- and 30% for ruptured TAAA treated by multibranch
comes for open repair. In 1 center, the operative mortality endovascular repair.
rate in 783 patients was 5.6%, with a low risk of SCI of 3. In patients with known or suspected connective tissue
2.0% and need for postoperative hemodialysis of 5.2%. disorders, such as Marfan syndrome, Loeys-Dietz syn-
Another center, whose operators used deep hypothermic drome, or vascular Ehlers-Danlos syndrome, open
circulatory arrest, reported an operative mortality rate of repair is recommended. Operative mortality rate is
6.8% with an SCI risk of <3% and postoperative hemodi- lower than in the general population undergoing open
alysis risk of 2.2%. TAAA repair, as is the incidence of major complica-
tions, such as stroke and SCI. Importantly, freedom
from aortic reintervention is excellent, as is long-term
1. In patients with ruptured TAAA, open repair can be survival. Conversely, data are lacking on complex
performed with low mortality by surgeons in centers endovascular repair of TAAA for patients with con-
with a Multidisciplinary Aortic Team. In a series of 100 nective tissue disorders. A small study of 17 patients
consecutive patients with ruptured TAAA, an operative treated by fenestrated-branched endovascular aortic
mortality rate of 14% and an SCI rate of 5% was ach- repair (FEVAR) had no mortality rate, 100% technical
ieved.5 Although the study population was replete with success, and 1 reintervention at mean follow-up of 34
comorbid conditions, the only risk factor remaining months.6 Endovascular repair may be reasonable in
significant after propensity matching was “shock” on patients who failed previous open repair or are
arrival to the hospital. Furthermore, 5-year actuarial considered high risk and have stent-grafts placed into
survival was 47.5%. Centers experienced in complex synthetic landing zones, or when used as a bridge to
endovascular repair may opt to use this technique. In a open repair in patients with hemodynamic instability.
national registry of 140 ruptured descending aneu- 4. Single- and multi-institution series of physician-
rysms, the operative mortality rate (10%) was good, but sponsored investigational device exempt trials have
there was a disappointingly high rate of stroke (14.7%), shown the promise of fenestrated and branched
SCI (9.6%), and need for reintervention within 30 days endovascular stent grafts. When performed by experi-
(19.7%). At a median follow-up of 17 months, actuarial enced surgeons, technical success may be achieved in a
5-year survival rate was 31.9%. These results were high percentage of cases (92%–99.6%) with low peri-
similar to those reported from a device registry. 1,5 operative mortality rate. At 1-year follow-up imaging,
Although complex endovascular repair of intact TAAA branch vessel patency was also good (96%–98%) and,
has shown promise in experienced hands and in select at 3 years, freedom from aortic-related death was 91%
centers, in the setting of TAAA rupture, the and overall survival 57%.15
6.5.4.3. TAAA Spinal Cord Protection
Recommendations for TAAA Spinal Cord Protection

1. In patients undergoing open TAAA repair who are at high risk for SCI, cerebrospinal fluid drainage is
1 A recommended to reduce the incidence of temporary SCI, permanent SCI, or both.1-7
2. In patients who experience delayed spinal cord dysfunction after either open or endovascular TAAA
1 B-NR repair, timely measures to optimize spinal cord perfusion and decrease intrathecal pressure are recom-
mended (Table 20). 1-4,8

SCI is a devastating complication of open and endo-
1. TAAA repair remains a formidable undertaking regard-
vascular thoracoabdominal aneurysm repair, with an
less of whether open or endovascular repair is per-
incidence rate of 2% to 15%, depending on aneurysm
formed. SCI, with either paraparesis or paraplegia, may
extent and cause, underlying patient comorbidities, ur-
be temporary or permanent and has a profoundly
gency of the procedure, and surgeon and center experi-
negative impact on short- and long-term survival as well
ence. Previous ACC/AHA guidelines did not address the
as quality of life after repair. Many techniques have been
issue other than to suggest higher-risk populations that
suggested to reduce the incidence of this significant
might benefit from adjuncts to reduce the incidence of
complication. Intraoperative management ranges from
SCI.9 The 2014 European guidelines assigned cerebrospi-
deep hypothermic circulatory arrest to left heart bypass
nal fluid drainage a I B recommendation to reduce the risk
to a “clamp-and-sew” technique, and support exists for
of SCI.10 However, data were limited at the time to sup-
each approach. Similarly, intraoperative and post-
port this recommendation, and an earlier RCT 11 had not
operative spinal cord neuromonitoring is not wide-
shown a benefit for cerebrospinal fluid drainage in TAAA
spread but has support that is institutionally based.
repair. A more recent RCT did show a significant reduc-
Other interventions have been also advocated as intra-
tion in SCI for a cohort undergoing repair of extensive
thecal papaverine to enhance spinal cord protection.13
TAAA (extent I and extent II) when cerebrospinal fluid
Cerebrospinal fluid drainage remains the only tech-
drainage was used. Additional nonrandomized data sup-
nique proven to reduce the incidence of perioperative
port this recommendation.
SCI. In an RCT examining the incidence of SCI in patients
Delayed SCI may occur up to 2 weeks after surgery. This
undergoing high-risk extent I and II TAAA repair, cere-
complication has a profound impact on short- and long-
10,12 brospinal fluid drainage was associated with a signifi-
term outcomes. Early recognition and aggressive
cant reduction in SCI compared with those having
management of SCI can lead to a return of lower extremity
surgery without cerebrospinal fluid drainage. Over the
function. The reinsertion of a cerebrospinal fluid drain is a
past decade, there are few centers performing open
key component to salvage lower extremity function.
TAAA repair without the aid of cerebrospinal fluid
Additional therapies, such as volume loading, increasing
drainage. Furthermore, patients undergoing endovas-
mean arterial pressure, and maximizing oxygen delivery
cular repair requiring extensive descending thoracic
to the cord through transfusion or supplemental oxygen,
aorta coverage or in the setting of a previous infrarenal
are also critical.
aneurysm repair also benefit from cerebrospinal fluid
drainage (nonrandomized). 6
2. In patients undergoing open TAAA repair, delayed
TABLE 20 Measures to Optimize Spinal Cord and
End-Organ Perfusion paraplegia may account for nearly 60% of all spinal
Cardioversion for tachyarrhythmias cord deficits encountered. Despite having an intact
Insertion of cerebrospinal fluid drain neurologic examination immediately after the proced-
Increase mean arterial pressure to >100 mm Hg

ure, patients can experience these delayed deficits
anytime in the first 2 weeks postoperatively. The re-
Transfuse to a hemoglobin >10 g/dL
ported incidence of delayed SCI is approximately 5%,
Volume resuscitation
nearly twice that of deficits recognized immediately
after surgery. Delayed deficits usually present in the improvement in their neurologic examination, with
setting of a hemodynamic insult (atrial fibrillation, 17% having complete resolution of their deficits.14 The
hypovolemia, hemorrhage, infection) and may be operative mortality rate for those with persistent SCI is
responsive to aggressive measures to optimize spinal nearly 3-fold higher than for those who recover (38%
cord perfusion (Table 20). Cerebrospinal fluid drainage versus 13%, respectively; P<0.001). In addition, 5-year
immediately reduces intrathecal pressure and in- survival is significantly worse (from 75% with a return
creases spinal cord perfusion pressure (spinal cord of function to 28% without; P<0.001).14
perfusion pressure equals mean arterial pressure minus
spinal cord fluid pressure).8,12,14 A significant propor- 6.5.4.4. TAAA Renal and Visceral Organ Protection
tion (57%) of patients with late deficits experience an
Recommendations for TAAA Renal and Visceral Organ Protection

1. In patients undergoing open repair of TAAA involving the renal arteries, cold blood or crystalloid renal
1 A perfusion is recommended to provide effective protection against renal injury.1-6
2. In patients undergoing open or endovascular TAAA repair who have end-organ ischemia or significant
1 B-NR stenoses from atherosclerotic visceral or renal artery disease, additional revascularization procedures are
recommended. 7
Synopsis dysfunction.7 Subsequently, cold blood perfusate

Postoperative renal dysfunction after open TAAA repair delivered to the renal arteries through occlusion or
has a significantly negative impact on short- and long- perfusion catheters was found to provide the same level
term mortality as well as quality of life. Efforts to reduce of renal protection as cold crystalloid perfusate during
renal injury during open TAAA repair include local organ open TAAA repair.5 The results of this second RCT
hypothermia with either cold crystalloid or cold blood- provided surgeons with 2 options for renal protection
based perfusate. when open TAAA repair requires renal artery
reconstruction.
2. In patients with renal or visceral artery stenoses or
1. Renal dysfunction after TAAA repair is defined as a ostial obstruction secondary to chronic or acute
doubling of the creatinine or the need for hemodialysis. dissection flaps, end-organ perfusion may be compro-
When this significant complication occurs, short- and mised. Improvement in perfusion to the celiac axis,
long-term survival is compromised, and the incidence SMA, and both renal arteries may be achieved by
of postoperative respiratory failure, SCI, and cardiac bypass, endarterectomy, or balloon angioplasty and
complications increase. To identify methods to reduce stent placement. Patency of target vessel revasculari-
the incidence of postoperative renal dysfunction, 2 zation strategies has been documented in small series
RCTs were performed comparing cold crystalloid renal of patients having open TAAA repair with a
preservation to normothermic blood perfusate and, “debranching” technique and in those undergoing
subsequently, cold blood perfusate. When compared endovascular TAAA repair.
with normothermic blood delivered into the renal ar-
teries directly from the left heart bypass circuit, the 6.5.5. Abdominal Aortic Aneurysms
delivery of cold crystalloid perfusate into the renal ar-
6.5.5.1. Access During Endovascular Repair of AAA
teries during open TAAA repair resulted in a 3-fold
reduction in the incidence of postoperative renal
Recommendation for Access During Endovascular Repair of AAA

1. In patients undergoing endovascular repair of AAA who have suitable common femoral artery anatomy,
1 B-R ultrasound-guided percutaneous access and closure is recommended over open cutdown to reduce oper-
ative time, blood loss, length of stay, time to wound healing, and pain.1,2
Synopsis too low to produce a difference in outcomes, in-

Increased availability of percutaneous closure devices vestigators found that, compared with open cutdown
and lower profile endovascular stent grafts have made for access, groins accessed and closed percutaneously
ultrasound-guided percutaneous access and closure more healed faster and patients reported less pain.1 Although
feasible. Two RCTs and a large national retrospective re- the PEVAR trial did not require ultrasound-guided
view showed favorable outcomes from percutaneous femoral access, it was routine in the PiERO trial.
common femoral artery access and closure such as Furthermore, a multicenter observational study of
reduced operative time, reduced blood loss, and common femoral artery access showed a significant
improved patient-centered outcomes, such as reduced decrease in groin hematomas with routine ultrasound-
pain. guided access. 4 Lastly, in an extensive comparison of
different closures using data from 13,087 patients in the
Vascular Quality Initiative registry, there was a signif-
1. The PEVAR trial showed the noninferiority of total icantly higher rate of cardiac complications (OR, 1.5;
percutaneous access and closure for EVAR for those 95% CI, 1.14–2.05) and 30-day mortality rate (OR, 1.56;
with suitable common femoral artery anatomy.3 In the 95% CI, 1.05–2.32) 2 in those undergoing cutdown versus
PiERO (Percutaneous femoral access in Endovascular percutaneous access. 2 Operative time, estimated blood
Repair versus Open femoral access) study, investigators loss, and length of stay were all significantly higher in
evaluated whether ultrasound-guided percutaneous those undergoing groin cutdowns compared with
access via the common femoral artery decreased the percutaneous access.
risk of surgical site infections compared with cutdown.
Although the incidence of surgical site infections was
6.5.5.2. Repair of Ruptured AAA
Recommendations for Repair of Ruptured AAA

1. In patients presenting with ruptured AAA who are hemodynamically stable, CT imaging is recommended
1 B-R to evaluate whether the AAA is amenable to endovascular repair.1-3
2. In patients presenting with ruptured AAA who have suitable anatomy, endovascular repair is recom-
1 B-R mended over open repair to reduce the risk of morbidity and mortality.1,4-6
3. In patients undergoing endovascular repair for ruptured AAA, local anesthesia is preferred to general
2a B-NR anesthesia to reduce risk of perioperative mortality.7-9
4. In patients with ruptured AAA, permissive hypotension can be beneficial to decrease the rate of
2a C-LD bleeding. 1,3,10-12
Synopsis reported to be as low as 18.5% after instituting an endo-

The mortality rate from ruptured abdominal aortic an- vascular repair-first strategy in at least 1 observational
eurysms (rAAA) is estimated to be 80% to 90%, with most series. 1 Initial randomized trials for endovascular repair
13
patients never reaching the hospital. For those who for rAAA (rEVAR) versus open repair generally showed no
present to a hospital, the historical mortality rate for open early survival benefit. However, shortcomings of these
repair was approximately 50%. With improved team or- trials raised questions about their applicability. 2,14,15
ganization, prompt diagnosis, and endovascular repair Longer-term studies of rEVAR, such as 3-year results
options, the mortality rate after repair for rAAA has been from the IMPROVE (Immediate Management of the Patient
With Rupture: Open Versus Endovascular Repair) trial, (hazard ratio, 0.57; 95% CI, 0.36–0.9).16 Contemporary
showed late survival benefit from rEVAR over open repair. observational studies showed significant survival
Many authors have evaluated institutional experience benefit from an endovascular approach to rAAA. For
with using rEVAR in anatomically suitable candidates and example, Wang et al6 used propensity-matched data
aimed to improve the process of care for rAAA by adopting from the Vascular Quality Initiative registry and
“rupture protocols” that include early imaging, permis- showed that rEVAR resulted in a lower 30-day mor-
sive hypotension, endovascular balloon occlusion under tality rate than open repair (21% versus 34%, respec-
fluoroscopy to reduce excessive bleeding, and a team- tively; P<0.001) and that mortality rates after rEVAR
based organization to facilitate immediate transfer of have been steadily decreasing since 2008. Other
patients to the operating room for prompt hemorrhage studies have corroborated this general decline in the
control and repair.1,3 rEVAR mortality rate and comparatively better post-
operative outcomes.4,17 Newer endovascular devices
have enabled treatments of rAAA that do not neces-
1. The IMPROVE trial was the first trial to evaluate a new sarily meet instructions for use criteria. However,
paradigm in evaluating rAAA.2 Specifically, patients caution should be exercised, because observational
who were hemodynamically stable were first trans- studies showed increased risk of perioperative death
ported to the radiology suite for CTA to assess whether and long-term complications when devices are used
their ruptured aneurysm was amenable to endovas- off-label in a rupture scenario. 18,19
cular repair or required open repair. This is in contrast 3. Patients presenting with rAAA often maintain
to a strategy of transport to the operating room for adequate BPs, in part because of the body’s catechol-
open surgery without preoperative imaging. The trial amine responses.20 However, once induced with gen-
did not identify any increased risk of death from a eral anesthesia, the loss of this physiologic response—
strategy of acquiring preoperative imaging and, coupled with anesthetic agents that can depress BP—
because of the different repair options available today, can lead to circulatory collapse. 21-23 General anesthesia
such assessments can help surgeons choose appro- has also been shown to have deleterious effects on
priate therapy based on patient aneurysm anatomy and inflammatory and body temperature regulation.24,25
clinical status. In contemporary practice, many pa- Subanalysis of the IMPROVE trial showed that pa-
tients will have a CT scan, although some of these tients with rAAA who underwent EVAR with only local
scans will not be ideally timed arterial phase imaging. anesthesia had lower risk of mortality compared with
Given that time is of the essence in rAAA repair, if a those who were treated under general anesthesia
patient’s CT scan provides enough anatomic informa- (adjusted OR, 0.27; 95% CI, 0.1–0.7). 7 Although the trial
tion to identify whether endovascular repair is was not designed and powered for this specific
feasible, another more dedicated CTA scan may add outcome, recent observational studies from large reg-
unnecessary delays to the patient’s care. istries have corroborated this finding.8,9
2. Although 3 clinical trials aimed to evaluate potential 4. Although there are no RCTs of outcomes specific to
survival benefit for rEVAR over open repair, none permissive hypotension in rAAA, data from the trauma
showed significant early benefit. However, trials literature evaluating fluid management in hemorrhagic
excluded patients who were hemodynamically unsta- shock show benefit in using a strategy of permissive
ble, thus excluding patients that may have benefitted hypotension.11,12 Many authors managing rAAA have
most from an endovascular approach. It should be similarly described maintaining low arterial pressures
noted, however, that the IMPROVE trial subsequently to decrease rate of bleeding in patients with rAAA.1,3,10
showed that between 90 days and 3 years, rEVAR had An SBP that allows a patient to maintain mentation,
superior survival rates compared with open repair typically between 60 and 90 mm Hg, is suggested.
6.5.5.3. Threshold for AAA Repair
Recommendations for the Threshold for AAA Repair

1. In patients with unruptured AAA, repair is recommended in those with a maximal aneurysm diameter
1 A of ‡5.5 cm in men or ‡5.0 cm in women. 1-6
2. In patients with unruptured AAA who have symptoms that are attributable to the aneurysm, repair is
1 B-NR recommended to reduce the risk of rupture. 7,8
3. In patients with unruptured saccular AAA, intervention to reduce the risk of rupture may be reasonable.9
2b C-LD
4. In patients with unruptured AAA and aneurysm growth of ‡0.5 cm in 6 months, repair to reduce the risk
2b C-LD of rupture may be reasonable. 1-5
Synopsis survival benefit for repair of aortic aneurysms

One of the most significant risk factors for continued measuring 4.0 cm to 5.4 cm. 1-5 Although long-term
aneurysm growth and rupture is the maximum diameter. outcomes in the UKSAT group seemed to show better
Thresholds for AAA repair must balance the expected risk survival rates in patients in the early open surgery
of rupture against the risk of operative intervention. group, this was thought to be attributable to higher
Historically, the risk of rupture was reported to be 0.5% to rates of smoking cessation in the early surgery group
5% for aneurysms <5 cm in maximum diameter, 3% to 15% compared with the surveillance group.2,3 Based on
for aneurysms 5 cm to 6.9 cm, and $30% for these data, balancing the risk of intervention versus
aneurysms $8 cm. 10 Multiple trials that are now >2 de- the risk of rupture, a threshold of $5.5 cm is acceptable
cades old evaluated the use of early repair of AAAs for men with infrarenal AAA. In the UKSAT study,
measuring 4.0 cm to 5.4 cm via open or endovascular which included more women than the previous
means. All found no survival benefit attributable to early studies, women were found to have higher rates of
repair and but did find an increased risk of subsequent aneurysm rupture and higher rates of aneurysm-
reintervention. These studies and others have found that related deaths than men.2,3 The mean maximum
rupture does occur at smaller diameters for women; thus, aneurysm diameter at rupture was 5.0 cm in women
size thresholds for men and women differ to account for and 6.0 cm in men. More recent data highlight a
these observed differences.6,11 Newer data highlight other different method for quantifying aneurysm rupture
considerations, such as aortic indexing, which may better risk by indexing aneurysm size to the BSA (ASI equals
predict aneurysm rupture risk. Lastly, although limited aneurysm diameter [cm]/BSA [m 2]); in women, ASI has
data exist for the natural history of saccular AAAs, avail- been shown to be more predictive of rupture risk than
able data suggest that their morphologic features may is maximum diameter. 12 Further research will help
make them more likely to become symptomatic, rupture clarify whether ASI is a better metric for aneurysm
at smaller diameters, or both than fusiform AAAs. repair thresholds than maximum diameter. 12
2. Approximately 6% to 22% of treated aneurysms are
symptomatic but unruptured. Symptoms that are
1. Clinical trials conducted in the late 1990s and early considered high risk for impending rupture include
2000s, including the UKSAT (UK Small Aneurysm Trial) pain in the back, abdomen, or flank, and sometimes
and ADAM (Aneurysm Detection and Management) radiating to the groin, which is attributable to the AAA.
trial for early open aneurysm repair and CAESAR Patients presenting with such symptoms should be
(Comparison of surveillance vs. Aortic Endografting for admitted to an ICU for arterial BP monitoring, tight BP
Small Aneurysm Repair) and PIVOTAL (Positive Impact control, medical optimization, and AAA repair, ideally
of endoVascular Options for Treating Aneurysm earLy) in 24 to 48 hours to reduce risk of free rupture. Other
trials for early endovascular repair, did not find a symptoms that warrant expedited, although not
necessarily urgent AAA repair, include tenderness to had diameters <5.5 cm and <4.5 cm, respectively. In
palpation overlying the AAA in the abdomen, back, or their 2017 guidelines on AAA,16 the Society for Vascular
flank, embolism (eg, blue toe syndrome) or compressive Surgery recommended elective repair of patients pre-
symptoms (eg, obstructive uropathy). Observational senting with saccular AAA, although size guidance is
studies show that patients treated for symptomatic lacking because of limited natural history data. Clearly,
aneurysms have higher mortality and morbidity rates the decision to intervene must be informed by the
than those treated electively.7,8 Although timing of patient’s individual anatomy.
repair of symptomatic aneurysms remains controver- 4. Pooled analysis from thousands of patients included in
sial, most studies have reported outcomes of symp- AAA surveillance studies from North America, Western
tomatic aneurysms repaired during a patient’s index Europe, and East Asia showed that, although aneurysm
operation, with some studies finding that performing growth is highly variable, growth rates range from 1.5
surgery on a nonemergency basis and potentially opti- mm/y to 2 mm/y for those with AAA of 3.0 cm to 3.9 cm
mizing patient’s cardiorespiratory status during their and from 3.3 mm/y to 5.7 mm/y in AAA of 4.0 cm to 5.9
hospitalization may be advantageous.8,13-15 cm at baseline. 17,18 The 4 major trials evaluating effi-
3. Saccular AAAs are rare and, consequently, there are cacy of early open and endovascular treatment of AAA
limited natural history data. In a Dutch registry of pa- for small aneurysms all excluded patients with aneu-
tients treated for fusiform and saccular AAAs, re- rysms that grew $7 mm in 6 months or >10 mm in 12
searchers found that saccular aneurysms appeared months, given concern for increased risk of rupture.
more common in women and were more likely to be Thus, balancing the risks, aneurysms with size in-
symptomatic at smaller sizes than fusiform aneu- creases of $0.5 cm in 6 months or $1 cm in 1 year are
rysms. 9 Of 7,659 patients with AAA, 6.1% had saccular considered to be rapidly growing and may warrant
AAA. Of patients with saccular aneurysms and acute consideration of repair.
presentation, 25% had diameters <5.5 cm, and 8.4%
had diameters <4.5 cm. In contrast, only 8.1% and 6.5.5.4. Open Versus Endovascular Repair of AAA
0.6% of patients with fusiform AAA presenting acutely
Recommendations for Open Versus Endovascular Repair of AAA

1. In patients with nonruptured AAA with low to moderate operative risk and who have anatomy suitable for
1 A either open or EVAR, a shared decision-making process weighing the risks and benefits of each approach is
recommended. 1-11
2. In patients undergoing elective endovascular repair for nonruptured AAA, adherence to manufacturer’s
1 B-NR instructions for use is recommended. 12-16
3. In patients with nonruptured AAA and a high perioperative risk, EVAR is reasonable to reduce the risk of
2a B-NR 30-day morbidity, mortality, or both. 9,10
4. For patients with nonruptured AAA, a moderate to high perioperative risk, and anatomy suitable for an
2a B-NR FDA-approved fenestrated endovascular device, endovascular repair is reasonable over open repair to
reduce the risk of perioperative complications. 10,11,17,18
Synopsis and consider patient preferences for surgical options

Options for repair of AAA have substantially grown when data support either approach. Historic RCTs evalu-
since the first description of open repair in 1952. 19 In ating outcomes of EVAR versus open repair showed an
particular, EVAR has made it possible to treat patients who initial survival advantage for EVAR that dissipates at
may have never qualified for open surgery because of different time intervals.1,3-8 Contemporary investigations
significant cardiopulmonary comorbidities, renal comor- have shown a steady decline in mortality rates for EVAR in
bidities, or both. With the abundance of options, clinicians general20 and a much larger perioperative survival benefit
must remain informed regarding empirical data that may from EVAR versus open repair. 9 However, similar to his-
favor one approach over another in a particular patient toric clinical trials, these survival benefits can dissipate
over time and must be weighed against suboptimal sur- instructions for use in elective AAA repair is discour-
veillance that can occur in those treated with EVAR, aged. Those who have been treated off instructions for
leading to higher rates of late rupture and associated use warrant closer follow-up because of higher rates of
death. 21 For repair of juxtarenal aneurysms using FDA- failure from endoleaks, graft migration, and late
approved fenestrated devices, available data show rupture.
similar findings (ie, an initial survival benefit that may 3. EVAR-2 (UK Endovascular Aneurysm Repair 2) was an
wane over time).10,11 RCT that evaluated outcomes of EVAR in high-risk
patients. Patients were enrolled if they were deter-
mined to be unfit for open surgery, with fitness
1. Pooled data from 7 RCTs evaluating all-cause death assessed using cardiac, respiratory, and renal criteria.23
after EVAR versus open surgery for infrarenal AAA In these patients, the trial initially showed that EVAR
repair show that the risk of perioperative mortality is did not improve survival compared with the control of
much lower in those treated with EVAR (OR, 0.36; 95% no intervention; however, more than a decade later,
CI, 0.2–0.66). This advantage persists at 6 months, af- those treated with EVAR had significantly lower
ter which survival from both approaches become aneurysm-related mortality (hazard ratio, 0.55; 95% CI,
equivalent. Moreover, after 8 years, those treated with 0.34–0.91). 24,25 Contemporary analyses of outcomes in
EVAR have a higher risk of aneurysm-related death high-risk patients show that perioperative death after
(hazard ratio, 5.12; 95% CI, 1.6–16.4), secondary inter- EVAR has markedly decreased (eg, 9% in EVAR-2
vention (hazard ratio, 2.1; 95% CI, 1.7–2.7), aneurysm versus 1.9% in the ACS national registry). 26 Further-
rupture (OR 5; 95% CI, 1.1–23.3), and death attributable more, in evaluating a propensity-matched Medicare
to rupture (OR 3.6; 95% CI, 1.9–6.8) compared with population, postoperative complications that are more
open repair. 22 Observational studies, such as the large likely to affect high-risk patients, such as myocardial
propensity-matched study evaluating EVAR and open infarction, pneumonia, acute renal failure, and need
repair in a Medicare population, found that the sur- for dialysis, were all significantly less likely to occur
vival advantage for EVAR lasted longer among older after infrarenal EVAR compared with open repair.9 In
9
patients. For complex repairs, a similar survival assessing which patients are “high risk” for elective
advantage is seen for fenestrated repair over complex AAA repair, risk calculators derived using data from the
open repairs in the first 30 days after surgery. More Vascular Quality Initiative and the Vascular Study
data are necessary to identify longer-term outcomes Group of New England can be helpful in informing
and to determine for which groups one approach may discussions with patients about repair options and
be more advantageous. Given the current clinical potentially identify patients for which even EVAR
equipoise, engaging the patient in a process of shared would be of prohibitively high risk. 27-29
decision-making is recommended, as further detailed 4. Recent observational studies aimed to compare out-
in Section 5, “Shared Decision-Making.” comes between open and endovascular repair for
2. Patient-specific anatomical characteristics of the aorta, complex aortic aneurysms. Using propensity score
such as neck diameter, length, and angulation, and matching, investigators found that perioperative mor-
iliac seal diameter, length, and vessel access, must all tality rates between patients undergoing open repair or
be considered in endovascular repair. Some observa- FEVAR were similar in those enrolled in the Vascular
tional studies show that treating aneurysms outside of Quality Initiatives registry (4.7% versus 3.3%, respec-
the manufacturer’s instructions for use increases fail- tively, P¼0.17).17 Evaluating data from the ACS, Var-
ure rates, resulting in increased risks of graft migra- kevisser et al found much higher odds of 30-day death
tion, endoleaks, late rupture, and deaths.12,13 For from open repair compared with FEVAR (OR, 4.9; 95%
example, Shanzer et al12 found that in a multicenter CI, 1.4–19). 10 The risk of immediate postoperative
retrospective study of >10,000 patients undergoing complications, such as myocardial infarction, acute
EVAR between 1999 and 2008, patients with AAA kidney injury, and the initiation of dialysis, is signifi-
treated with devices off instructions for use had cantly higher after open complex repair compared with
significantly higher rates of sac enlargement. More FEVAR.11,17,18 However, rates of late reintervention are
13
recently, Herman et al found that any deviation from higher after FEVAR,11,18 as are the rates of persistent
instructions for use increased risk of graft-related renal impairment11 and 3-year mortality rate
adverse events (hazard ratio, 1.8; 95% CI, 1.05–3.1). A (excluding perioperative deaths) (hazard ratio, 1.7; 95%
meta-analysis of 17 studies found that patients treated CI, 1.1–2.6).17 Thus, similar to infrarenal repair, FEVAR
with noninstructions for use higher overall mortality may be most beneficial for the moderate- to high-risk
rates (hazard ratio, 1.2; 95% CI, 1.02–1.42; P¼0.03).14 surgical candidates who are more likely to experience
Given these findings, in most patients, treating off perioperative complications.
6.5.5.5. Treatment of Concomitant Common Iliac Aneurysms
Recommendations for the Treatment of Concomitant Common Iliac Aneurysms

1. For patients with asymptomatic small AAA and concomitant common iliac artery aneurysm(s) ‡3.5 cm,
1 C-LD elective repair of both abdominal and iliac aneurysms is recommended. 1-4
2. When treating common iliac artery aneurysms or ectasia as part of AAA repair, preservation of at least
1 B-NR 1 hypogastric artery is recommended, if anatomically feasible, to decrease the risk of pelvic ischemia.5,6
Synopsis 1 patient presented with a ruptured internal iliac artery

The prevalence of common iliac artery aneurysms in aneurysm of #3 cm, and 4 individuals’ iliac aneurysms
the presence of AAA has been reported to be as high as ruptured at diameters #4 cm. Recently published data
20% to 40% in surveillance studies. 1,2 In patients with from the Dutch Surgical Aneurysm Audit showed that
both aortic and iliac aneurysms, it is common for an iliac of the 857 patients with treated iliac artery aneurysms,
aneurysm to reach a size appropriate for elective repair the median iliac artery aneurysm size at elective repair
before the AAA does. Although no randomized studies for was 4.3 cm, while ruptured iliac aneurysms had a me-
iliac aneurysm repair size thresholds exist, in large case dian diameter of 6.8 cm at presentation.
series and registry reports, rupture of iliac aneurysms at 2. In a meta-analysis of studies reporting exclusion or
diameters <4 cm is rare.3,7 Thus, a repair threshold of 3.5 preservation of the internal iliac artery, Kouvelos et al 5
cm seems reasonable to balance procedural risks with found an increased pooled occurrence of buttock
rupture risk. Furthermore, to achieve adequate AAA claudication in those undergoing unilateral (27%) or
repair, repair of iliac artery ectasia or aneurysms often bilateral (36%) internal iliac artery exclusion. In a
may be required. Consideration of pelvic perfusion is of separate meta-analysis, Bosanquet et al 6 found similar
great importance when managing concomitant iliac dis- rates of buttock claudication, as well as a 10% occur-
ease. In such cases, there is a high risk of ischemic com- rence of erectile dysfunction in men. Other ischemic
plications from exclusion of internal iliac arteries that can events, such as spinal, bowel, and gluteal ischemia,
lead to buttock claudication, bowel ischemia, and erectile were rare, occurring at a rate of <1%.6 Another
5,6
dysfunction. For some patients, adequate treatment of consideration in treating aorto-iliac disease is the risk
diseased iliac arteries cannot be accomplished without of late intervention from growth of ectatic or aneu-
internal iliac artery sacrifice. Thus, individualized treat- rysmal iliac arteries. In a retrospective analysis of
ment plans with shared decision-making are important prospectively collected data, Gibello et al 4 found that
when treating aorto-iliac aneurysm disease. in patients with AAA undergoing EVAR, after a mean
follow-up of 6.2 years, those with common iliac arteries
of $18 mm in diameter had a significantly higher rate of
1. In a large single-center case series by Huang et al, 8 438 type Ib endoleaks (7.2% versus 3.2%; P¼0.01) and late
patients with common iliac artery aneurysms were reinterventions (19% versus 11.8%; P¼0.01), leading to
observed for an average of 3.7 years. Eighty-six percent higher odds of composite EVAR failure (OR, 1.8; 95% CI,
of patients had current or previously treated AAA. 1.2–2.7) and need for reintervention (OR, 1.9; 95% CI,
Common iliac artery aneurysms grew at an average rate 1.15–3.3). Hassen-Khodja et al10 and Sala et al 9 found
of 2.9 mm/y, and no iliac aneurysm #3.8 cm ruptured. that, after open repair of AAA, common iliac arteries
A multinational retrospective review of patients with of $18 mm in diameter tended to dilate over time,
internal iliac artery aneurysms found that 41.7% of in- warranting consideration of bifurcated grafting rather
dividuals had a concomitant AAA. Of 63 patients, than aorto-aortic tube grafting.9,10
6.5.6. Surveillance After Aneurysm Repair

6.5.6.1. Surveillance After TAA Repair
Recommendations for Surveillance After TAA Repair

1. In patients treated with TEVAR, surveillance imaging with CT is recommended after 1 month and
1 B-NR 12 months and, if stable, annually thereafter. 1-5
2. In patients treated with TEVAR, longitudinal surveillance with MRI is a reasonable alternative to CT for
2a B-NR reduction of long-term radiation exposure or avoidance of an iodinated contrast allergy. 6-9
3. In patients treated with open repair of the thoracic aorta without residual aortopathy, surveillance im-
2a B-NR aging with a CT or MRI within 1 year postoperatively and then every 5 years thereafter is reasonable. 10-14
4. In patients treated with open repair of the thoracic aorta who have residual aortopathy or abnormal
2a C-EO findings on surveillance imaging, annual surveillance imaging is reasonable.
Synopsis TEVAR.2,17 A 6-month follow-up study may be useful in

The role of surveillance imaging after thoracic aneu- detecting a delayed retrograde type A aortic dissection.
rysm repair is to identify complications of the repair or 2. MRI has some advantages over CT, including the
monitor for progression of residual aortic pathology. CT is avoidance of ionizing radiation and iodinated intrave-
generally the preferred imaging modality for surveillance nous contrast administration.7,8 However, MRI is
7,15
imaging after TEVAR ; MRI, although generally more limited by its higher cost, longer acquisition times,
limited by metallic artifact, is a reasonable alternative. lower resolution, and limited visualization of metallic
Open repair of the thoracic aorta is durable.2,5,10-14 In stent graft components and adjacent structures. MRI
patients undergoing TEVAR, there is a higher incidence of has a potential growing role, particularly in patients
complications and reintervention compared with patients who are middle aged or younger, in whom the conse-
undergoing open repair 2,4,5,10-12; TEVAR complications quences of lifelong surveillance in terms of contrast-
can include endoleak (see Section 2.6, “Classification of induced nephropathy and cumulative radiation dose
Endoleaks”), retrograde type A aortic dissection, stent- should be considered.9
graft migration, stent-graft fracture or collapse, and an 3. Open repair for any segment of the thoracic aorta has
increase in aortic size. 6,7 Complications of open repair proven to be durable in extended follow-up. 10,11,13,14,19
that can be detected by surveillance imaging include graft Treatment failure after open repair of either the prox-
infection and anastomotic pseudoaneurysm.10,16 Addi- imal or distal thoracic aorta requiring reintervention
tionally, after both open repair and TEVAR, patients may ranges from 1% to 7% in long-term (10-year) follow-
develop progressive aneurysmal dilation of adjacent or up.10-12 In patients without a genetic syndrome or re-
remote aortic segments. sidual aortopathy shown on a postoperative imaging,
surveillance can be done at longer intervals.
4. The appropriate frequency surveillance imaging in the
1. Use of TEVAR is associated with reintervention rates presence of abnormal findings has neither been stud-
ranging from 7% to 23%. 1,2,4,5 In the Gore TAG study,17 ied nor validated but, in such cases, annual surveil-
there was an 11% incidence of endoleak 18 at 30 days, lance imaging is typical. Patients requiring
6% at 1 year, and 9% at 2-year follow-up after reintervention have a higher incidence of HTAD.10,16
6.5.6.2. Surveillance After AAA Repair
Recommendations for Surveillance After AAA Repair

1. In patients with AAA treated with EVAR, baseline surveillance imaging with CT is recommended at
1 B-NR 1 month postoperatively1,2 ; if there is no evidence of endoleak or sac enlargement, continued surveillance
with duplex ultrasound at 12 months and then annually thereafter is recommended. 1,3,4
2. In patients with AAA treated with EVAR who are undergoing annual surveillance imaging duplex ultra-
2a C-LD sound, additional cross-sectional imaging with CT or MRI of the abdomen and pelvis every 5 years
postoperatively is reasonable.5-8
3. In patients with AAA treated with EVAR and abnormal findings (Table 21) on any surveillance duplex
2a C-LD ultrasound, additional cross-sectional imaging with CT or MRI is reasonable.9
4. In patients with AAA treated with complex EVAR, a modified surveillance imaging plan that combines
2a C-LD cross-sectional imaging and duplex ultrasound of target vessels is reasonable.10,11
5. In patients with AAA who have undergone open repair, surveillance imaging with CT or MRI of the
2a C-LD abdominopelvic aorta within 1 year postoperatively and then every 5 years thereafter is reasonable.5,6
Synopsis aneurysmal disease in the adjacent visceral aorta or iliac

The role of routine surveillance after EVAR is to iden- arteries.17 Patients with a history of AAA are also at risk of
tify endoleak, sac growth, endograft migration, or developing an aortic aneurysm in a noncontiguous
endograft failure. Although the initial surveillance in- location. 18
tervals after EVAR were at 1 month, 6 months, and 12
months postoperatively to be consistent with surveillance
imaging intervals used in FDA-sponsored device trials, 1. The incidence of late aortic rupture after EVAR is >5%
more recent data suggest that the 6-month interval can be through 8 years of follow-up.3 Significant risk factors
eliminated if no concerning findings are observed on the for rupture include endoleak with associated aneurysm
1-month imaging (Table 21).1,2 sac enlargement. 19,20 Endoleaks may be present for
CT is the gold standard for follow-up imaging after 10% to 17% of EVAR at 30 days postoperatively.1,2 In
EVAR, but it is expensive, exposes the patient to ionizing patients without early (30-day) endoleak, the inci-
radiation, and requires the use of iodinated contrast that dence of new endoleak at 6 and 12 months post-
is potentially nephrotoxic. 12,13 Duplex ultrasound, with or operatively is similar.1 Earlier detection of an endoleak
without contrast enhancement, has been shown to be at 6 vs. 12 months is not associated with improved
specific for the detection of endoleaks after EVAR9,14 and long-term outcomes.1,2
15
complex EVAR ; however, ultrasound is limited in its 2. Stent graft fracture and migration is a long-term
ability to detect stent migration, fracture, or noncontig- complication after EVAR that occurs in 3% to 4% of
uous aneurysms. MRI has high diagnostic accuracy for patients by 4 years postoperatively.7,8 Duplex ultra-
16
endoleaks but must be accompanied by a plain abdom- sound has been shown to be specific for the detection
inal radiograph to assess for endograft stent fracture, of endoleaks after EVAR 9,14,15 but is limited in its
because MRI cannot accurately visualize the metallic ability to detect stent migration, fracture, or new
stent struts. noncontiguous aneurysms.
The role of routine surveillance after open AAA repair is 3. Duplex ultrasound is 95% accurate for measuring aortic
to prevent late aneurysm rupture and aneurysm-related aneurysm sac diameter and 100% specific for the
death by detecting para-anastomotic and new aneu- detection of type I and type III endoleaks (Figure 11)
rysms. Para-anastomotic aneurysms can occur after open after EVAR but is insufficient for detecting type II
AAA repair as a result of anastomotic disruption, leading endoleaks9 or for characterizing anatomy related to
to pseudoaneurysm formation or progression of stent graft migration or failure.
surveillance of para-anastomotic aneurysms after open

Abnormal Findings on Duplex Imaging After
TABLE 21 AAA repair.18
EVAR That Should Prompt Additional Imaging
Aneurysm sac enlargement
Any endoleak
Stent graft fracture 7. ACUTE AORTIC SYNDROMES

Stent graft migration
Stent graft separation 7.1. Presentation
EVAR indicates endovascular abdominal aortic aneurysm repair. AAS, although uncommon, are associated with life-
threatening complications and a mortality rate as high
as 1% to 2%/h if the AAS is not rapidly identified and
appropriate therapy is not instituted promptly.1 The
4. Duplex ultrasound has been shown to be a useful mo- diagnosis of AAS can be challenging, however, because
dality for surveillance of target branch vessels 11 after the presenting symptoms overlap with other more com-
FEVAR. However, complex EVAR involving stenting mon emergency department complaints.
of $1of the renovisceral vessels is at higher risk of type Although the classic textbook description of AAS is of
III endoleak than standard EVAR 10 and may benefit acute “tearing” or “ripping” pain, patients more
from routine cross-sectional imaging for surveillance commonly report the abrupt onset of severe “sharp” or
of fenestration sites, branch junctions, and adequacy “stabbing” pain in the chest or back (and sometimes
of flow in the renal and mesenteric arteries. 21 abdomen), maximal at the start, that sometimes radi-
5. Para-anastomotic aneurysms after open AAA repair ates.2-5 Depending on the extent of aortic involvement,
tend to occur late, with estimated incidence rates of patients may present with various additional signs and
1%, 6%, and 27% to 35% at 5, 10, and 15 years post- symptoms (Table 22). Recording a careful history of the
operatively, respectively.5,6 Late aortic aneurysms in presenting symptoms is essential, as is obtaining a
noncontiguous arterial segments from the initial aortic detailed family history of TAAs, genetic aortopathies,
repair have been reported in 45% at a mean of 7 years aortic dissection, or unexplained sudden death.
postoperatively.18 As a result, the Society for Vascular BP should be measured in both arms and both lower
Surgery and the European Society of Cardiology have extremities, to exclude a BP differential resulting from an
both recommended surveillance imaging every 5 years AAS. One should auscultate for the murmurs of aortic
after open AAA repair.22 No data support the use of 1 stenosis, perhaps indicating an underlying BAV, and AR,
cross-sectioning imaging modality over another for the which commonly accompanies type A aortic dissection.
TABLE 22 Signs and Symptoms of AAS
Clinical Signs and Symptoms Cause

Asymmetric blood pressure (>20 mm Hg) between limbs Compromise of branch artery flow
Bowel ischemia or gastrointestinal bleed Malperfusion of the celiac or superior mesenteric artery
Dysphagia Compression of the esophagus
Dyspnea Compression of trachea or bronchus, congestive heart failure from aortic regurgitation, or cardiac tamponade
Hemoptysis Vascular rupture into lung parenchyma
Hoarseness Compression recurrent laryngeal nerve
Horner’s syndrome Compression of sympathetic chain
Myocardial ischemia or myocardial infarction Coronary artery involvement by dissection or compression by aneurysm
New murmur of aortic regurgitation Incomplete aortic valve closure secondary to leaflet tethering by the dilated aorta or cusp prolapse
because of dissection into the aortic root
Oliguria or hematuria (gross) Malperfusion of 1 or both renal arteries
Paraplegia Spinal malperfusion attributable intercostal artery involvement
Lower extremity ischemia Malperfusion of iliac artery
Shock Cardiac tamponade, hemothorax, frank aortic rupture, acute severe aortic regurgitation, severe myocardial
ischemia
Shortness of breath Pericardial effusion, congestive heart failure from acute severe aortic regurgitation, or hemothorax
Stroke symptoms Carotid or vertebral artery involved
Superior vena cava syndrome Compression of the superior vena cava
Syncope Carotid artery involvement or cardiac tamponade
AAS indicates acute aortic syndrome.

7.2. AAS: Diagnostic Evaluation (Imaging, Laboratory Testing)
Recommendations for AAS: Diagnostic Evaluation (Imaging, Laboratory Testing)
1. In patients with a suspected AAS, CT is recommended for initial diagnostic imaging, given its wide
1 C-LD availability, accuracy, and speed, as well as the extent of anatomic detail it provides. 1-5
2. In patients with a suspected AAS, TEE and MRI are reasonable alternatives for initial
2a C-LD diagnostic imaging. 1-6
Synopsis CT is widely available at all hours in the emergency

A plain chest x-ray is neither sufficiently sensitive nor department and is quick to perform. Not only does
specific for AAS to be used to be diagnostic, but certain it diagnose the underlying AAS, it also shows the
radiographic findings (Table 23) may raise suspicion of full extent of the dissection and, in some cases,
aortic dissection or suggest an alternate diagnosis for the the entry tear site. CT can detect the presence and
patient’s symptoms, in particular when there is previous mechanism of aortic branch vessel involvement as
radiography that shows the changes to be new in the in- well as vessel patency, signs of malperfusion, pericar-
terval. 1,2,7 Fortunately, CT, TEE, and MRI are all highly dial effusion and hemopericardium, periaortic or
accurate for the diagnosis of AAS.3 Aortography is rarely mediastinal hematoma, and pleural effusion. Use
used given its invasive nature and significantly lower of electrocardiographic-synchronized CT techniques
sensitivity than the other imaging modalities.8 Acute should be considered when there is a need to accu-
aortic dissection risk scoring systems (eg, aortic dissec- rately depict mediastinal structures (eg, proximal
tion detection risk score [AAD-RS] or aorta simplified aorta, coronary ostia). When IMH is present, the extent
score [AORTAs]) can aid in the diagnostic evaluation of and thickness of the hematoma can be documented
patients presenting with AAS (Table 24 and Table 25) 5,9-12 and, when PAUs are present, the presence of and size of
but have not been uniformly adopted.4 No biomarkers pseudoaneurysms can be easily defined.
are considered diagnostic, although in patients with a low 2. In general, the choice of the initial imaging modality
previous probability of AAS a nonelevated D-dimer (<500 should be based on the patient’s history and clinical
ng/mL) makes the diagnosis unlikely. Consequently, presentation, the specific clinical questions to be
integrating a low aortic dissection risk score and a low D- answered, and the institutional availability, experi-
dimer may be a useful strategy to exclude the diagnosis of ence, and expertise with each of the diagnostic imaging
AAS.13 techniques.6 In certain clinical circumstances, for
1. Although the sensitivity and specify of CT, MRI, and

TEE are all high,3 CT has become the preferred mo-
dality for evaluating most patients with suspected AAS. Aortic Dissection Detection Risk Score (ADD-RS)
TABLE 24
Items 5,14
High-Risk Pain High-Risk Examination

High-Risk Conditions Features Features
TABLE 23 Plain Chest X-Ray Suggestive of Aortic n Marfan syndrome or Chest, back, or n Pulse deficit or systolic
Dissection 2 other connective tissue abdominal pain blood pressure differential
disease described as:
Signs of Aortic Dissection on Chest X-Ray Findings n Abrupt onset
n Family history of aortic n Focal neurologic deficit
Mediastinal widening disease (with pain)
n Known aortic valve n Severe in n Murmur of aortic regurgita-
Disruption of the normally distinct contour of the aortic knob
disease intensity tion (new, with pain)
”Calcium sign,” which appears as a separation of the intimal calcification from the n Recent aortic n Ripping or tearing n Hypotension or shock state
aortic wall of >5 mm manipulation in quality
Double density appearance within the aorta n Known thoracic aortic
aneurysm
Tracheal deviation to the right
For each risk category, 1 point is assigned if $1 risk factors are present. Consequently, the total
Deviation of the nasogastric tube to the right
ADD-RS will range from 0 to 3. An ADD-RS of 0 points is low risk; 1 point is moderate risk; and 2 to 3
Reprinted with permission from Strayer et al.2. points is high risk. Adapted with permission from Hiratzka et al.5 Copyright 2010, American Heart
Association, Inc., and American College of Cardiology Foundation.
identifying complications of AAS, such as AR or peri-

TABLE 25 Aorta Simplified Score (AORTAs) 11 Pretest
cardial effusion and tamponade. TEE is preferred to
Probability Assessment Score
TTE, however, because of its higher sensitivity and
Clinical Item Points
better anatomic resolution; TEE can be performed at
Hypotension/shock 2
the bedside in the emergency department or, alterna-
Aneurysm 1
tively, once the patient is in the operating room. MRI is
Pulse deficit 1
most commonly the third-choice modality, given that it
Neurologic deficit 1 is not readily available, requires skilled interpretation,
Severe pain 1 and has longer acquisition times, as well as the fact it is
Sudden-onset pain 1 challenging to provide clinical care to potentially un-
The patient is given the number of points corresponding to each clinical item that is stable patients while in an MRI scanner. Consequently,
positive in the patient’s presentation. The points are summed, and a total score of 0 to 1 MRI is most often used as a follow-up imaging modality
point is low-probability of aortic dissection, where a total of $2 points is high proba-
bility. Reprinted with permission from Morello et al.11 in patients in which there is diagnostic uncertainty.
Nevertheless, MRI may be the study of choice in the
acute setting for a stable patient with a contraindica-
example, patients with a history of an iodinated
tion to iodinated contrast.
contrast reaction or patients who are too unstable to
travel to the radiology suite, CT may not be preferred.
Echocardiography (TEE/TTE) is an alternative. TTE is
noninvasive, can be performed at the bedside, and may 7.3. Medical Management of AAS
be helpful in eliciting the diagnosis of AAS and quickly 7.3.1. Acute Medical Management of AAS
Recommendations for Acute Medical Management of AAS

1. In patients presenting to the hospital with AAS, prompt treatment with anti-impulse therapy with
1 B-NR invasive monitoring of BP with an arterial line in an ICU setting is recommended as initial treatment to
decrease aortic wall stress.1-5
2. Patients with AAS should be treated to an SBP <120 mm Hg or to lowest BP that maintains adequate end-
1 C-LD organ perfusion, as well as to a target heart rate of 60 to 80 bpm. 3,6
3. In patients with AAS, initial management should include intravenous beta blockers, except in patients
1 B-NR with contraindications.2,5,7
In those with contraindications or intolerance to beta blockers, initial management with an intravenous
2a B-NR non-dihydropyridine calcium channel blocker is reasonable for heart rate control. 1,2,5
4. In patients with AAS, initial management should include intravenous vasodilators if the BP is not well
1 C-LD controlled after initiation of intravenous beta-blocker therapy. 8
5. Patients with AAS should be treated with pain control, as needed, to help with hemodynamic
1 C-EO management.
Synopsis (in patients with type B aortic dissection), or both; med-

Patients presenting with AAS need to be treated ical therapy includes aggressive heart rate and BP man-
promptly to prevent acute and chronic complications. In agement as well as pain control. Studies have shown that,
all patients with AAS, immediate medical therapy is beyond surgical and endovascular treatment, medical
indicated while considering urgent surgical (in patients therapy has an important role in decreasing long-term
with type A aortic dissection), endovascular intervention aorta-related adverse events.1,4,9-11 Beta blockers and
intravenous vasodilators are the medications most 3. Intravenous beta blockers have been the mainstay of
commonly studied for the initial treatment of patients acute medical treatment, and studies reporting bene-
with AAS, with the goal of decreasing aortic wall stress. 2,8 fits over the long term and emphasizing the importance
A recent large study showed that angiotensin-converting of continuing this therapy at the time of hospital
enzyme inhibitors (ACEIs) and ARBs are beneficial in the discharge to improve clinical outcomes.1-3,5,7,9 Caution
long-term management of hypertension in patients with should be used in patients with contraindications to
aortic dissection.5 Statins are used routinely in patients beta blockers (eg, acute AR, heart block, or brady-
after aortic dissection, although the evidence is not very cardia). In patients who are intolerant to beta blockers,
robust.12 intravenous non-dihydropyridine calcium channel
blockers (ie, verapamil or diltiazem) are typically used
for initial treatment.2
1. There are no randomized studies that have evaluated 4. Intravenous vasodilators are useful adjunctive therapy
different medical treatments in the treatment of AAS, for intravenous beta blockers but should be avoided as
although extensive clinical experience has established initial treatment (before starting beta blockers or cal-
the current standard of anti-impulse therapy. This is cium channel blockers), given the potential for
usually accomplished with a combination of intrave- compensatory tachycardia. 8,9
nous beta blockers (eg, esmolol, metoprolol, and 5. Pain related to AAS can trigger a rise in heart rate and
labetalol) and vasodilators (eg, nicardipine, clevidi- BP, so treating the pain symptoms can help to control
pine, and sodium nitroprusside) with the goal of the patient’s BP and heart rate. Intravenous opiates are
reducing both heart rate and BP to decrease aortic wall particularly efficacious in this situation. Intravenous
stress.2-5,7,8,11 nonsteroidal anti-inflammatory drugs, such as ketor-
2. Small, single-center studies have highlighted the olac, may not be suitable because of the risk of
importance of reducing heart rate to 60 to 80 bpm and inducing hypertension as well as adverse renal effects.
SBP to <120 mm Hg. Experts believe that the lowest BP
that does not compromise end-organ function should
7.3.2. Subsequent Medical Management of AAS
be targeted.3,11
Recommendation for Subsequent Medical Management of AAS

1. In patients with AAS, it is recommended to treat with long-term beta blockers (unless contraindicated) to
1 B-NR control heart rate and BP to reduce late aortic-related adverse events. 1-7 Additional antihypertensive
agents (particularly ARBs and ACEIs) should be added, as necessary, to adequately control BP.
Synopsis mouse models of Marfan syndrome as well as case

Patients with AAS with surgical or endovascular treat- control studies in Marfan syndrome and other inherited
ment need continued and long-term medical management. aortopathy patients in the GenTAC (Genetically Trig-
Controlling hypertension has consistently been shown to gered Thoracic Aortic Aneurysms and Cardiovascular
decrease aorta-related adverse events. Recent studies Conditions) registry showed deleterious effects of long-
have shown long-term benefit with specific BP agents such term calcium channel blocker use and, consequently, it
as beta blockers, ACEIs, and ARBs. may be best to avoid these agents in patients with
Marfan syndrome unless necessary to achieve BP
Recommendation Supporting Text
control.8
1. Long-term oral antihypertensive regimens that included
beta blockers, ACEIs, and ARBs have shown to improve 7.4. Surgical and Endovascular Management of Acute Aortic
long-term outcomes in patients with AAS treated with Dissection
1-4
both surgical and endovascular treatments. Although The primary goals of open surgical or endovascular stent-
calcium channel blockers showed some benefit in pa- graft repair for acute aortic dissection are to prevent (or
tients with type B aortic dissection, further studies in treat) aortic rupture, prevent retrograde extension of the
F I G U R E 2 1 Acute Aortic Dissection: Malperfusion Treatment Options
AoD indicates aortic dissection; and TEVAR, thoracic endovascular aortic repair.
dissection into the aortic root, prevent antegrade propa- the specific features and constraints of the patient’s aortic
gation of the dissection into distal yet undissected seg- and branch vessel anatomy (Figure 21).
ments, and alleviate malperfusion syndromes. Acute 7.4.1. Acute Type A Aortic Dissection
aortic dissection management strategies are therefore
7.4.1.1. Initial Surgical Considerations in Acute Type A
“complication specific,” guided by the patient’s signs and
Aortic Dissection
symptoms, the presence or absence of complications, and
Recommendations for Initial Surgical Considerations in Acute Type A Aortic Dissection

1. In patients presenting with suspected or confirmed acute type A aortic dissection, emergency surgical
1 B-NR consultation and evaluation and immediate surgical intervention is recommended because of the high risk
of associated life-threatening complications. 1,2
2. In patients presenting with acute type A aortic dissection, who are stable enough for transfer, transfer
2a B-NR from a low- to a high-volume aortic center is reasonable to improve survival. 3,4
3. In patients presenting with nonhemorrhagic stroke complicating acute type A aortic dissection, surgical
2a B-NR intervention is reasonable over medical therapy to reduce mortality and improve neurologic outcomes. 5,6
Synopsis occurring in those undergoing surgery 8 to 12 hours

Acute type A aortic dissection is a life-threatening after diagnosis. 16 Patients presenting with clinical in-
condition because of potential sequelae, including dicators of severe physiologic compromise (shock,
rupture that causes cardiac tamponade, acute severe AR neurologic deficits, malperfusion, myocardial
that causes heart failure or shock, compromised coronary ischemia) mandate the most immediate consideration
artery ostia causing myocardial ischemia, or malperfusion for repair as the only potential option for survival.
causing end-organ ischemia or infarction, all of which can 2. Patients with acute type A aortic dissection who pre-
all be fatal. Suspected or diagnosed acute type A aortic sent with hemodynamic stability have an unpredict-
dissection warrants urgent surgical evaluation, because able course because of the inability to predict eventual
the mortality rate of medical management alone is 2 to 3 rupture. Although some studies have suggested that
times that of surgical intervention.1 Data from IRAD night-time surgery is associated with a higher mortality
showed that from 1995 to 2013, the surgical mortality rate rate, 17,18 other studies have shown no diurnal differ-
decreased from 25% to 18%, while the medical mortality ence in outcomes,19,20 and all studies have shown no
rate remained unchanged at 57%. Surgical intervention difference with weekend surgery. Surgeon and center
mitigates the immediate risk of aortic rupture/tampo- experience and resource availability should be
nade, corrects AR and myocardial ischemia, and reestab- considered to ensure optimal outcomes. Despite an
lishes flow to malperfused vessels. inherent delay in the start time of surgery, transfer
Nevertheless, the benefits of surgery must be weighed from low- to high-volume hospitals (one that
against the risks of the surgery itself (ie, a demanding, performs $7 aortic root, ascending aorta, or transverse
complex operation in patients who often are physiologi- arch aortic dissection repairs per year),3 as part of
cally compromised). Universally recognized risk factors regionalization of care, can result in significantly
that increase the surgical mortality rate include shock and improved outcomes.3
tamponade, neurologic or visceral malperfusion, and 3. In patients with cerebral malperfusion, survival is su-
preoperative myocardial ischemia.7-9 Although age is a perior with surgery; in patients with acute type A aortic
risk factor, elderly patients still benefit from surgery, with dissection and an acute stroke, the mortality rates of
superior immediate and midterm outcomes compared surgical versus medical management are 25% to 27%
with medical therapy.10,11 Short- and midterm outcomes versus 76%, respectively.5,21 Even more strikingly,
12,13
can be equivalent to younger populations, with cir- Estrera et al showed that patients with acute type A
culatory collapse being the primary predictor of long-term aortic dissection who had presented with stroke had an
survival.14 In patients with significant contraindications operative mortality rate of only 7% and showed no
to surgery, including frailty, clinical judgment may worsening of neurologic status postoperatively.6
determine that the risk-benefit ratio favors medical Although their study and others,6,22 have emphasized
management. the timeliness of the aortic repair in stroke patients,
with a cutoff of w5 to 10 hours (after which neurologic
outcomes declined), Fischbein et al23 found no associ-
1. The potential sequelae of acute type A aortic dissec- ation between postoperative neurologic improvement
tion, including myocardial infarction, acute AR, cardiac and time from onset of neurologic symptoms to sur-
tamponade, aortic rupture, and end-organ malperfu- gery. IRAD data revealed that cerebrovascular accident
sion, are associated with high rates of morbidity and and coma resolved in 84% and 79% of patients,
mortality. Given the acuity, unpredictability, and fi- respectively, despite mean times to surgery of 12.3 and
nality of such events, immediate evaluation for surgi- 13.8 hours, respectively.24 It should be noted, however,
cal intervention is warranted to reverse any ongoing that in 1 recent report of 11 patients with acute type A
physiologic compromise and mitigate the risk of fatal aortic dissection and complete occlusion of an internal
events. The mortality rate of unoperated acute type A carotid artery, all died from cerebral edema and her-
aortic dissection is 1%/h,15 and the time intervals be- niation, regardless of management25; consequently,
tween symptom onset, diagnosis, and surgery have a this particular subset of patients may not benefit from
significant effect, with the highest mortality rate surgical intervention.
7.4.1.2. Management of Malperfusion
Recommendations for Management of Malperfusion

1. In patients with acute type A aortic dissection presenting with renal, mesenteric, or lower extremity
1 B-NR malperfusion, it is recommended to proceed to immediate operative repair of the ascending aorta.1,2
2. In patients with acute type A aortic dissection presenting with clinically significant mesenteric (celiac,
2a C-LD SMA) malperfusion, either immediate operative repair of the ascending aorta or immediate mesenteric
revascularization via endovascular or open surgical intervention by those with this expertise before
ascending aortic repair is reasonable.3-6
Synopsis malperfusion, extremity malperfusion, uncomplicated

Imaging evidence of malperfusion is present in as mesenteric malperfusion, or all of them is present. 9
many as 25% of patients with acute type A aortic dissec- This strategy rapidly mitigates the risk of aortic
tion but should be distinguished from clinical evidence of rupture and corrects any associated coronary malper-
end-organ ischemia, which is often referred to as mal- fusion, AR, and the sequelae of tamponade. After
perfusion syndrome (Table 26). Malperfusion syndrome is central aortic repair, any residual malperfusion should
associated with a mortality rate of 30.5%, compared with be assessed with secondary interventions, as needed.
a mortality rate of only 6.2% in those without malperfu- 2. Mesenteric malperfusion is one of the worst compli-
sion syndrome.2 Mortality rate correlates with the number cations of acute type A aortic dissection, with an
of branch artery vessels involved1 as well as the number associated mortality rate of 63.2%. 1 Consequently, such
7
of malperfused organs. The combination of pulse deficits patients are often managed with medical therapy
(a marker of malperfusion) and hypotension should alone; yet, in IRAD, the nearly one-third of patients
prompt timely interventions to reestablish vital organ with mesenteric ischemia who were treated without
perfusion, because early reperfusion predicts survival. 8 intervention had an in-hospital mortality rate of 95%. 10
The traditional approach to reestablish branch vessel For patients with acute type A aortic dissection who
perfusion has been via central aortic repair (ie, at the present with clinical evidence of mesenteric ischemia,
proximal aortic tear site). However, cardiac and visceral some centers3,4 have advocated early direct reperfu-
malperfusion portend extremely poor outcomes given the sion strategies (whether via endovascular or open
high mortality rate associated with irreversible organ abdominal surgery 11 ), before central aortic repair; other
damage. More recent series showed potential to improve centers continue to advocate for the traditional strat-
outcomes by establishing end-organ perfusion using egy of central aortic repair first.1,2 Currently, data are
endovascular means, before open central aortic repair limited to help define the best strategy. In IRAD, a
(with the timing of subsequent open repair decided on a surgical and hybrid strategy appears to have superior
case-by-case basis). 5,8 These procedures may be per- outcome to medical or endovascular therapy alone. An
formed in a hybrid operating room if the requisite re- institution series of endovascular therapy first showed
sources and personnel are available. a low aortic repair operative mortality rate of 2.1%;
however, only 58% of the cohort underwent open
repair, with 24% dying from organ failure and 13% from
1. In the presence of malperfusion, operative mortality aortic rupture. Moreover, an endovascular therapy first
rate correlates with the number of malperfused organs. approach requires expertise in fenestration, to treat
Central aortic repair as the primary strategy to restore dynamic obstruction, and branch stenting, to treat
perfusion has reasonable results when renal static malperfusion.5
TABLE 26 Clinical Evidence of Malperfusion (“Malperfusion Syndrome”)
End Organ Clinical Findings

Cardiac Electrocardiographic changes of ischemia or infarction, troponin elevation, myocardial dysfunction
Cerebral Stroke and neurologic deficits, coma and altered mental status
Spinal Paraplegia
Mesenteric Abdominal pain, bowel ischemia, lactic acidosis, elevation of liver function test results
Renal Acute kidney injury, oliguria
Extremity Loss of pulses in $1 extremity, sensory or motor dysfunction

7.4.1.3. Surgical Repair Strategies in Acute Type A Aortic

Dissection
Recommendations for Surgical Repair Strategies in Acute Type A Aortic Dissection

Aortic Repair Strategies
1. In patients with acute type A aortic dissection and a partially dissected aortic root but no significant aortic
1 B-NR valve leaflet pathology, aortic valve resuspension is recommended over valve replacement.1-5
2. In patients with acute type A aortic dissection who have extensive destruction of the aortic root, a root
1 B-NR aneurysm, or a known genetic aortic disorder, aortic root replacement is recommended with a mechanical
or biological valved conduit. 6-9
In selected patients who are stable, valve-sparing root repair may be reasonable, when performed by
2b C-LD experienced surgeons in a Multidisciplinary Aortic Team.10,11
3. In patients with acute type A aortic dissection undergoing aortic repair, an open distal anastomosis is
1 B-NR recommended to improve survival and increase false-lumen thrombosis rates. 12-15
4. In patients with acute type A aortic dissection without an intimal tear in the arch or a significant arch
1 B-NR aneurysm, hemiarch repair is recommended over more extensive arch replacement.16-18
5. In patients with acute type A aortic dissection and a dissection flap extending through the arch into the
2b C-LD descending thoracic aorta, an extended aortic repair with antegrade stenting of the proximal descending
thoracic aorta may be considered to treat malperfusion and reduce late distal aortic complications. 19,20
Perfusion and Cannulation Strategies
6. In patients with acute type A aortic dissection undergoing surgical repair, axillary cannulation, when
2a B-NR feasible, is reasonable over femoral cannulation to reduce the risk of stroke or retrograde
malperfusion. 21,22
7. In patients with acute type A aortic dissection undergoing surgical repair who require circulatory arrest,
2a B-NR cerebral perfusion is reasonable to improve neurologic outcomes. 23-25
8. In patients with acute type A aortic dissection undergoing surgical repair, direct aortic26,27 or innominate
2a B-NR artery28 cannulation with imaging guidance is reasonable as an alternative to femoral or axillary can-
nulation. 29-31
Synopsis to aortic root management is based on pathology and

To reduce the risk of late aortic complications, surgical general condition. Younger patients are more likely to
resection should include the tear site, any aneurysmal have proximal extension or root involvement and may
aorta, and the proximal-most extent of the dissection. A have greater potential for late complications, given their
nonresected primary tear is a risk factor for reoperation.32 longer life expectancy. VSRR has been described with
A more extensive replacement that involves the aortic excellent outcomes, but long-term reoperative risk is a
root, arch, or both adds operative complexity, ischemic concern.33
time, and potentially circulatory arrest time but may Similarly, untreated aortic arch or descending thoracic
reduce the risk of future aortic dilation, aortic insuffi- aortic tissue may be at risk of aneurysmal enlargement
ciency, or repeat dissection. An individualized approach and the need for reintervention, particularly with acute
type A aortic dissection that extends into the descending and only antegrade perfusion after aortic perfusion—
thoracic aorta. An open distal anastomosis allows direct were used, compared with the absence of any of these
arch inspection for intimal tears and resection of the components. Open distal anastomosis is also associ-
lesser curve of the arch (ie, hemiarch technique) without ated with higher rates of complete false-lumen
increased operative death. 12,13,34 In-hospital death is thrombosis.13
lower with hemiarch repair than with total arch replace- 4. Single-institution study findings that total arch
ment. Antegrade stenting of the proximal descending replacement (TAR) is safe and promotes aortic remod-
thoracic aorta may promote false-lumen thrombosis and eling47,48 have not been resulted in larger studies.
35-37
positive remodeling, but long-term aortic-related GERAADA (German Registry for Acute Aortic Dissection
data are scarce. Type A) found a trend toward lower mortality rates
Involvement of the aortic arch by the aortic dissection with hemiarch versus TAR (18.7% versus 25.7%;
can influence both interventional strategies and clinical P¼0.07); higher rates of excessive bleeding and
outcomes. Various interventional approaches, such as rethoracotomy in the total arch group; and, in patients
extended open arch replacement (with or without a without preoperative neurologic deficits, lower mor-
frozen elephant trunk),44 hybrid techniques, or endo- tality rates for hemiarch than TAR (14.1% versus 24%,
vascular stenting have been described. 38-40 Aortic arch respectively; P¼0.02). 49 A n STS database study of 12
exclusion with emerging endovascular stents graft de- years of acute type A aortic dissection repairs showed
vices is a field in evolution. significantly lower operative death with hemiarch than
with TAR (16% versus 27%; P<0.001).50 Two meta-
analyses have found significantly lower mortality
1. Most single-center retrospective studies and an IRAD rates with partial compared with TAR.16,18 Across 3
study found no difference in perioperative mortality or meta-analyses, the long-term freedom from aortic
survival when comparing root replacement with a reoperation does not appear to be necessarily superior
more limited root repair or supracommissural with TAR. 16-18
2,5,7,41,42
replacement. However, a standardized and 5. Comparative data on use of antegrade stenting of the
structured algorithmic approach showed a mortality descending thoracic aorta in the setting of surgical
rate of only 8.1% with aortic valve resuspension as the acute type A aortic dissection repair are limited. In
preferred approach, whenever feasible, compared with several noncomparative meta-analyses, the mortality
23.1% with root replacement. 43 Studies on freedom rate was w8% to 12%, the stroke rate was 5% to 7%, and
from reoperation are mixed,1,7,41,44-46 but 2 meta- the SCI rate was 2% to 3.5%.36,51,52 False-lumen
analyses have shown excellent long-term durability thrombosis rates appear favorable,35 but the reinter-
of aortic valve resuspension, with reoperation rates vention rate was not zero, and the long-term benefit
1.4% to 2.1% per patient-year and low thromboembo- for aortic reoperation or aortic-related mortality
lism and bleeding rates (1.4%/patient-year).3,4 remained undefined. In a series that included 19 pa-
2. An aneurysmal root at the time of acute type A aortic tients with DeBakey type I acute type A aortic dissec-
dissection repair is at long-term risk of progressive root tion and clinical malperfusion, antegrade stenting was
dilation, secondary aortic insufficiency, and the need associated with resolution of malperfusion in 16 pa-
for reoperation. Specifically, an aortic root diameter of tients (84.2%). 19 In patients requiring total arch
>4.5 cm has been shown to be a risk factor for late replacement, a frozen elephant trunk has higher
reintervention.6 A valved conduit is one option for root adverse events in acute type A aortic dissection than in
replacement but, if the aortic valve leaflet quality is elective repairs. The stent length should be <15 cm
good, the aortic insufficiency is primarily attributable and, to avoid SCI, coverage should not extend to T8. 53
to sinus dilation, and the surgeon is experienced in 6. An STS database study50 and 2 meta-analyses21,22 have
VSRR, a VSRR may be reasonable for younger patients. found an increased risk of stroke and short-term mor-
3. In the development of the IRAD risk score, right hem- tality with femoral compared with axillary cannula-
iarch replacement was an independent predictor for a tion. However, femoral cannulation is more expedient
favorable surgical outcome.15 NORCAAD (Nordic Con- and is considered the primary arterial site in patients
sortium for Acute Type A Aortic Dissection) found that with hemodynamic instability mandating immediate
the open-distal technique was associated with better cannulation, or with anatomic features precluding
short- and midterm survival than the clamp-on tech- axillary cannulation. If initial femoral cannulation is
nique, although it was also associated with greater required for these reasons, it is recommended to cen-
rates of cerebrovascular complications. 12 Lawton et al14 trally canulate after the distal anastomosis has been
found superior survival when all 3 components—no completed, to maximize reestablishment of true lumen
cross-clamp use, deep hypothermic circulatory arrest, flow.
7. Some form of cerebral perfusion, whether antegrade or its safety relative to axillary cannulation is controver-
retrograde, has been shown to improve neurologic sial,57 because stroke rates with this technique are as
outcomes, when compared with deep hypothermic high as 20% in some series.29 Rosinski et al found that
23
circulatory arrest alone. Antegrade cerebral perfusion patients undergoing direct aortic cannulation were
is associated with both lower long-term mortality rates more hemodynamically unstable and had more
and neurologic dysfunction rates. Unilateral and extended repairs; however, even in multivariable
bilateral antegrade cerebral perfusion appear to have logistic regression, it was associated with a higher
similar outcomes, except in cases of prolonged circu- risk of stroke (OR, 2.3; 95% CI, 1.05–5.1) Further, cere-
latory arrest (>30–50 minutes), in which case bilateral bral perfusion by some means other than axillary
antegrade cerebral perfusion may be advanta- perfusion will be required for longer circulatory arrest
geous.24,54-56 cases. Innominate artery cannulation is another option
8. Several series for surgery for acute type A aortic that provides access for antegrade cerebral perfusion
dissection have reported direct aortic cannulation us- and appears to be safe in acute type A aortic
ing a TEE-guided Seldinger technique. When per- dissection.58-60
formed correctly, this technique has the benefit of
rapid establishment of cardiopulmonary bypass with
7.4.2. Management of Acute Type B Aortic Dissection
true lumen flow. However, when the patient is stable,
Recommendations for the Management of Acute Type B Aortic Dissection

1. In all patients with uncomplicated acute type B aortic dissection, medical therapy is recommended as the
1 B-NR initial management strategy. 1-3
2. In patients with acute type B aortic dissection and rupture or other complications (Table 27), intervention
1 C-LD is recommended.4-6
In patients with rupture, in the presence of suitable anatomy, endovascular stent grafting, rather than
1 C-EO open surgical repair, is recommended.
In patients with other complications, in the presence of suitable anatomy, the use of endovascular
2a C-LD approaches, rather than open surgical repair, is reasonable.4-6,7
3. In patients with uncomplicated acute type B aortic dissection who have high-risk anatomic features
2b B-R (Table 28), endovascular management may be considered.8,9
Synopsis been a large RCT comparing open versus endovascular

Although acute complicated type B aortic dissection repair for either complicated or uncomplicated type B
historically has been treated with open repair, endovas- aortic dissection.
cular therapy has largely supplanted open repair given
lower morbidity and mortality rates. Additionally, optimal
medical management was associated with a 30-day mor- 1. Those patients with type B aortic dissection generally
tality rate of 10% and midterm mortality rate of approxi- have better survival than those with type A aortic
mately 30%. The introduction of endovascular techniques dissection. In the acute uncomplicated setting, medical
has resulted in significantly lower morbidity and mortality management is the first mode of therapy for type B
rates when compared with optimal medical management, aortic dissection. A review of the IRAD database showed
reported in small randomized trials including ADSORB overall in-hospital mortality rate of 13%, with those
(Acute Dissection: Stent graft OR Best medical therapy) 8 requiring open repair having higher mortality rates
and INSTEAD (Investigation of Stent Grafts in Patients compared with those managed with optimal medical
with Type B Aortic Dissection) 10 ; to date, there has not management and percutaneous intervention (32.1%
TABLE 27 Consensus Features of Complicated Acute Type B Aortic Dissection
Feature Comment
Aortic rupture1 This can be either free or contained (including hemothorax, increasing periaortic hematoma, or both; or mediastinal
hematoma) and should be addressed promptly.
Branch artery occlusion and malperfusion2 Complete or partial occlusion of a major branch, with or without clinical evidence of ischemia; this includes visceral, renal,
and peripheral arterial branches.
Extension of dissection3 Extension of the dissection flap either distally or proximally (ie, retrograde type A dissection)
Aortic enlargement Progressive enlargement of the true, false, or both lumens while in the acute phase may require prompt intervention.
15
Intractable pain
Uncontrolled hypertension15
versus 9.6% versus 6.5%, respectively; P<0.0001).1 Fenestration may be required if TEVAR alone does
Without intervention, risk factors for early death not correct the malperfusion, and visceral or renal ar-
include shock, evidence of malperfusion, and age 1-3 tery stenting may also be required. When intervention
and can be grouped together with uncontrollable hy- is an emergency, TEVAR has a significantly lower
pertension, pain, and continued growth or extension of morbidity and in-hospital mortality rates compared
the dissection as a complicated type B aortic dissection. with open repair, with the greatest advantage among
2. Patients presenting with complicated acute type B older patients.12
aortic dissection (Table 27), or developing such features 3. With medical management of uncomplicated type B
after initial presentation, have an increased risk of aortic dissection still having a 30-day mortality rate of
morbidity and death, and urgent or emergency inter- 10% and a decreased long-term survival, interest re-
vention may be required. For rupture, rapid coverage mains in determining if early endovascular interven-
of the affected region of the descending aorta may be tion might reduce the risk of downstream complication
lifesaving and does not preclude subsequent further or negative aortic remodeling, particularly in patients
endovascular or open repair. This is an important with high-risk features. In the ADSORB trial,8 which
consideration in those patients with genetically trig- compared optimal medical management vs. optimal
gered aortic diseases (eg, Marfan syndrome, Loeys- medical management plus TEVAR, there were no early
Dietz syndrome). Cambria et al11 reviewed the out- deaths in either group and, at 1-year follow-up, there
comes for AAS managed with TEVAR compared with was just 1 death in the TEVAR group. TEVAR was su-
historic controls and found a 1-year survival rate of 79% perior to optimal medical management alone with
for acute type B aortic dissection treated endovascu- significant differences in incomplete or no false-lumen
larly. A subsequent single-arm study of patients thrombosis, aortic dilation, and rupture, but the pri-
treated with TEVAR found a 30-day mortality rate of mary clinical benefits are unknown. In the INSTEAD-XL
8% and 1-year survival rate of 88%. 4 The VIRTUE (Investigation of Stent-grafts in Aortic Dissection)
Registry investigators5 found a benefit to early inter- trial,13 in patients with uncomplicated type B aortic
vention but with reintervention rates of 20% to 39%. dissection, prophylactic TEVAR plus optimal medical
The RESTORE Patient Registry had similar results.6 was associated with improved 5-year aorta-specific
TABLE 28 High-Risk Features in Uncomplicated Acute Type B Aortic Dissection 9
High-Risk Imaging Findings
Maximal aortic diameter >40 mm
False-lumen diameter >20–22 mm
Entry tear >10 mm
Entry tear on lesser curvature
Increase in total aortic diameter of >5 mm between serial imaging studies
Bloody pleural effusion
Imaging-only evidence of malperfusion
High-Risk Clinical Findings
Refractory hypertension despite >3 different classes of antihypertensive medications at maximal recommended or tolerated doses
Refractory pain persisting >12 h despite maximal recommended or tolerated doses
Need for readmission

survival and delayed disease progression. As the long- with an increased need for future intervention are
term mortality rate for type B aortic dissection that is summarized in Table 28.9
managed medically and is strongly related to aortic
events, the findings from the ADSORB and INSTEAD-XL
trials appear promising, but larger trials with longer-
7.5. Management of IMH
term data are still needed. What remains unknown is
the optimal timing for TEVAR. 14 Features associated
Recommendations for Management of IMH

1. In patients with complicated (Table 29) acute type A or type B aortic IMH, urgent repair is recom-
1 B-NR mended. 1-3
2. In patients with uncomplicated acute type A IMH, prompt open surgical repair is recommended. 1,4-6
1 B-NR
In selected patients with uncomplicated acute type A IMH who are at increased operative risk and do not
2b C-LD have high-risk imaging features (Table 30), an initial or expectant approach of medical management
may be considered. 6-12
3. In patients with uncomplicated acute type B IMH, medical therapy as the initial management strategy is
1 B-NR recommended. 1-3,13
4. In patients with type B IMH who require repair of the distal aortic arch or descending thoracic aorta
2a C-LD (zones 2-5) and have favorable anatomy, endovascular repair is reasonable when performed by surgeons
with endovascular expertise. 2,14
5. In patients with type B IMH who require repair of the distal aortic arch or descending thoracic aorta
2a C-LD (zones 2-5) and have unfavorable anatomy for endovascular repair, open surgical repair is reasonable.2,3
6. In patients with uncomplicated type B IMH and high-risk imaging features (Table 30), intervention may
2b C-LD be reasonable. 13-16
Synopsis aortic dissection, or aortic rupture; alternatively, the he-

Aortic IMH is a distinct pathologic entity from aortic matoma can sometimes be resorbed.3 Of patients pre-
dissection and PAU. It is characterized by hemorrhage senting with AAS, the proportion who have IMH varies
within the media of the aortic wall and may occur with or based by region, with reports of 6% to 23% in North
without intimal disruption. Radiographically, IMH ap- America and Europe1,6 versus 26% to 44% in Asia.4,5,12
pears as a high-attenuation crescentic or circumferential The management strategy for IMH balances patient
thickening of the aorta on noncontrast imaging, with comorbidities, the differing lethality of type A and type B
absence of blood flow through a false lumen on contrast IMH, mortality and death associated with open or endo-
imaging. IMH occurs more commonly in the descending vascular repair in the different segments of the aorta, and
thoracic aorta (60%) than in the ascending aorta (30%) or the risk of aortic-related complications with medical
aortic arch (10%).1 Classification is the same as is used for management. Prospective randomized comparative
acute aortic dissection. Symptoms at presentation are studies are lacking, and most series and registries are
similar to aortic dissection, but patients tend to be older limited by small sample sizes. For recommendations
and more often have hypertension and atherosclerosis. 1,2 regarding management of IMH in association with PAU,
Malperfusion can occur but less frequently than in aortic see Section 7.6, “Management of Penetrating Atheroscle-
dissection. 1,2 IMH can progress to aortic enlargement, rotic Ulcer (PAU).”
Recommendation-Specific Supportive Text TABLE 29 Features of Complicated IMH
1. IMH, especially type A IMH, can be a lethal condition, Feature

complicated by rupture at presentation in 18% and, in n Malperfusion
that setting, associated with 100% mortality rate n Periaortic hematoma
without surgical intervention. 3 The features of com- n Pericardial effusion with cardiac tamponade
n Persistent, refractory, or recurrent pain
plications of IMH are summarized in Table 29.
n Rupture
2. A nonoperative strategy for type A IMH is associated
with a mortality rate as high as 40%, according to the IMH indicates intramural hematoma.
findings of the IRAD.1 Progression to aortic dissection,

rupture, or other aorta-related adverse events occurs in
14% to 37% of patients, with most events occurring decision-making with the patient should include dis-
within the acute or subacute phase. 12,17,18 In-hospital cussion regarding need for an extended hospital stay of
mortality (1%–27%)1,4,6 and mid- and long-term sur- 2 to 3 weeks, including $3 days in the ICU on bedrest,
vival4-6 after operative repair for type A IMH are with perhaps $5 CTAs during the hospitalization for
reasonable and comparable to or better than survival close monitoring because of the dynamic disease pro-
rates reported for type A aortic dissection. There are cess and moderate to high risk of progression to aortic
varied approaches to timing of surgery, with low dissection and rupture.
mortality rate achieved with strategies of repair within 3. Type B IMH may have a more benign prognosis than
24 hours 5 and slightly delayed repair (between 24 and type A IMH, resulting in relatively low in-hospital
72 hours), when feasible.6 The slight delay may confer mortality rate (4%–6%) with medical management
an advantage by allowing the hematoma to form and and 9% mortality rate at 1-year follow-up. 1,2 A strategy
the tissue quality of the aorta to improve. In addition, of medical management for type B IMH with surgical
the extra time can allow for further diagnostic evalu- intervention for severe recurrent symptoms or radio-
ation, optimization of comorbidities, or clearance of graphic worsening on follow-up was associated with
novel oral anticoagulant medications, which may acceptable long-term survival. 3 Intervention, whether
improve outcomes. Delay is only reasonable in stable surgical or endovascular, has associated mortality and
patients. The experience with successful medical morbidity. Although significantly less dissection and
management of type A IMH is mostly from Japan, Ko- rupture may be observed during follow-up with
rea, and China, all reporting outcomes better than TEVAR, compared with optimal medical therapy, this
those reported in North America and Europe; the dif- may 17 or may not 13,14 translate into improved aortic-
ferences might be related to genetic or environmental related outcomes.
factors that affect IMH natural history, so the Asian 4. Literature supporting endovascular treatment of IMH
results may not be generalizable to other ethnic or is limited mainly to experience with TEVAR for IMH in
geographic patient populations. The approach varies the setting of PAU, or TEVAR for mixed type B AAS
from initial medical management with planned including IMH; the perioperative mortality rate for
“timely” (ranging from within a few days to before treatment of acute IMH ranges from 0% to 29%. Of
discharge) surgery to expectant medical management note, in PAU with IMH or IMH with ulcer-like projec-
with surgical intervention only for complications or tion, endovascular treatment can be guided by the
disease progression. 7-12 One meta-analysis showed focal lesion. IMH with multiple ulcer-like projections
acceptable pooled proportion of an all-cause in-hospi- may require more extensive treatment length. Favor-
tal mortality rate of 7% and 30-day mortality rate of able anatomy for TEVAR would include ideally normal
15% 8 with initial medical management. Another meta- aorta at both proximal and distal landing zones or, at
analysis, comparing upfront surgery to initial medical least at the proximal landing zone, as outward tension
management with “timely” surgery, showed no sig- of the stent graft transferred to abnormal aortic wall
nificant difference in short-term survival (although an can lead to stent-induced new entry tear and subse-
overall operative approach to type A IMH did show a quent aneurysmal degeneration or aortic dissection. In
survival benefit over medical therapy alone).9 For pa- general, stent graft oversizing usually does not exceed
tients at increased operative risk (eg, advanced age, 10%, and balloon aortoplasty at the landing zones is
poor baseline renal function, coronary artery disease), avoided. The experience with TEVAR for retrograde
medical management may therefore be an option. type A IMH associated with a distal intimal defect (ie,
There are several high-risk imaging features (Table 30) distal arch or descending thoracic aorta) is limited to
that predict poor outcome (death, need for surgical small case reports and series. With the higher inci-
intervention, or both) with this strategy. Shared dence of atherosclerotic disease in patients with IMH
TABLE 30 High-Risk Imaging Features of IMH
For Type A IMH For Type B IMH
n Maximum aortic diameter >45–50 mm 18,20 n Maximum aortic diameter >47–50 mm 15,20
n Hematoma thickness $10 mm 4 n Hematoma thickness $13 mm 15
n Focal intimal disruption with ulcer-like projection involving n Focal intimal disruption with ulcer-like projection involving the descending thoracic
ascending aorta or arch18,21 aorta if it develops in acute phase15,16
n Pericardial effusion on admission18 n Increasing or recurrent pleural effusion19,22
For Both Type A and Type B IMH
n Progression to aortic dissection19

n Increasing aortic diameter21,22
n Increasing hematoma thickness21,22
IMH indicates intramural hematoma.
compared with aortic dissection, adequate vessel outpouching of contrast arising from the lumen of the
diameter for endovascular access should be deter- aorta in the setting of IMH with no associated athero-
mined as well. sclerotic plaque. FID is more specifically defined by its
5. In the IRAD experience for type B IMH, open surgical communicating orifice measuring >3 mm, while tiny
repair was performed in 5%, endovascular repair in 7%, intimal disruption has a communicating orifice #3
and a hybrid approach in 1%, with no difference in mm.15 FID occurs in 32% of type B IMH and signifi-
1
results. Good outcomes have been reported for open cantly predicts cardiovascular- or aorta-related death
repair,3,19 despite the more invasive approach. Open and aorta-related events,15,16,18 especially when it de-
surgical repair may be preferable when IMH extends to velops in the acute, rather than chronic, phase. 15 Tiny
the proximal landing zone of anticipated endovascular intimal disruptions are lower risk and considered a
coverage, the aortic diameter at the proximal or distal benign finding.16 As 40% of patients can develop FID
extent of planned coverage is too large to accommo- that was not present on the initial study, 15 early sur-
date existing stent graft sizes, the hematoma or aneu- veillance imaging can help identify patients at risk for
rysm extends into the aortic arch and circulatory arrest complications. Table 30 summarizes these and other
would facilitate resection of diseased aorta, or endo- high-risk imaging features of IMH.
vascular access for stent deployment is anticipated to
be inadequate.
6. High-risk imaging features may be present on admis-
7.6. Management of PAU
sion or may develop in the acute, subacute, or chronic
phases. Ulcer-like projections and focal intimal 7.6.1. PAU With IMH, Rupture, or Both
disruption (FID) are both terms that describe a focal
Recommendations for PAU With IMH, Rupture, or Both

1. In patients with PAU of the aorta with rupture, urgent repair is recommended.1-3
1 B-NR
2. In patients with PAU of the ascending aorta with associated IMH, urgent repair is recommended. 1-3
1 B-NR
3. In patients with PAU of the aortic arch or descending thoracic aorta with associated IMH, urgent repair is
2a C-LD reasonable. 1-3
4. In patients with PAU of the abdominal aorta with associated IMH, urgent repair may be considered. 4
2b C-LD
Synopsis rate of rupture is 33% to 75%,2,3 and progression to

A PAU is an atherosclerotic lesion of the aorta with aortic dissection is associated with a high mortality
ulceration that penetrates the internal elastic lamina and rate.1
allows hematoma formation within the media of the 3. PAUs with type B IMH that are managed conservatively
aortic wall. 5 PAUs may progress to AAS with IMH forma- are associated with a high risk of disease progression to
tion, aortic dissection, or rupture.1,2,6 PAU with IMH is true aortic dissection or rupture. 1,2 In a small retro-
associated with a high risk of short-term disease prospective analysis of patients presenting with PAUs and
gression,1 particularly when localized to the ascending type B IMH, 3 of 17 patients (17.6%) who were managed
aorta (ie, Stanford type A).1,2 Data on outcomes for PAU conservatively died from progression of disease to
with descending thoracic and abdominal aorta (ie, Stan- aortic rupture at a mean of 9.3 days.1 In contrast, there
ford type B) IMH are limited to small retrospective re- was 1 death among 14 patients (7.1%) who underwent
views but suggest significant early disease progression open (n¼8) or endovascular (n¼6) aortic repair for PAU
among patients treated with medical management.1,2 with type B IMH.1 These data support early interven-
PAUs tend to affect elderly patients with severe athero- tion of PAU in the setting of type B IMH in patients who
sclerotic disease and other comorbidities that put them at are reasonable surgical candidates.
high surgical risk even with endovascular interventions, 4. The natural history of PAU of the abdominal aorta with
so the risk of repair must be weighed against the risk of associated IMH is not well described, but low
severe morbidity and patient life expectancy when mak- procedure-related and 30-day mortality rates have
ing decisions about appropriate management. been described in several small series and case reports
of both the endovascular and surgical treatment of
abdominal aorta PAUs. 7 In a literature review of 298
1. PAU with rupture that is not treated with intervention published cases of PAU affecting the abdominal aorta,
is associated with a high mortality rate (of 5 of 17 pa- most authors (62.0%) reported endovascular stent graft
tients who presented with PAU with rupture who did repair as the treatment of choice, followed by open
not undergo repair, none survived).3 In contrast, in 1 surgical repair (35.4%) and conservative management
small series, most patients with PAU with rupture (2.6%).7
treated by open or endovascular therapy survived to
hospital discharge. 1
7.6.2. Isolated PAU
2. PAU of the ascending aorta is uncommon; however,
when it occurs, and in concert with IMH, the incidence
Recommendations for Isolated PAU

1. In patients with isolated PAU who are symptomatic and have persistent pain that is clinically correlated
1 B-NR with the radiologic findings, repair is recommended. 1-3
2. In patients with isolated PAU who are asymptomatic but have high-risk imaging features (Table 31),
2b C-LD elective repair may be considered. 1,2,4
Synopsis and frequent surveillance imaging to assess for disease

Isolated PAUs are those without associated IMH, aortic progression.4
dissection, or saccular aneurysm. Symptomatic isolated Asymptomatic isolated PAUs are increasingly diag-
PAUs may herald a developing peri-ulcer hematoma, IMH, nosed incidentally because of the increasing use of CTA.
or both and are more likely to progress or result in rupture Several series that reported mid or long-term outcomes
than asymptomatic PAUs. 5 For patients who present with of retrospective institutional data suggest that isolated
a symptomatic PAU but whose symptoms resolve with PAUs have radiographic progression in up to 30% of
goal-directed therapy or patients who are poor operative patients.1-3,6,7 High-quality data evaluating thresholds for
candidates at increased risk for morbidity and death from surgical repair are limited, but retrospective data have
repair, medical management has been pursued, with early shown that PAUs with a diameter of $13 mm to 20 mm or
TABLE 31 High-Risk Imaging Features of PAUs F I G U R E 2 2 Dimensions of Penetrating Atherosclerotic Ulcers
Feature
n Maximum PAU diameter $13–20 mm 1

n Maximum PAU depth $10 mm 1
n Significant growth of PAU diameter or depth
n PAU associated with a saccular aneurysm 5
n PAU with an increasing pleural effusion1
PAU indicates penetrating atherosclerotic ulcer.
depth of $10 mm (Figure 22) are closely associated with

disease progression.1 Significant growth rates are not well
defined and depend on the size of the patient, his or her
aortic anatomy, and the presence of high-risk features
associated with PAU (Table 31).
1. Symptomatic PAUs are associated with a high risk of

(A) Maximal aortic diameter at ulcer site diameter (from ulcer across to
early disease progression.2,3,5,7,8 In a small series of 25 opposite aortic wall). (B) Depth of intramural blood pool. (C) Length of
patients presenting with symptomatic PAU managed intimal defect at ulcer site. (D) Width of intramural blood pool.
medically, 30% had disease progression on surveillance Adapted from Gifford et al,11 Copyright 2016, with permission from
imaging at a mean of 18 months follow-up, including Elsevier, Inc., and from Cho et al9 Copyright 2004 with permission
from the Society for Vascular Surgery.
expansion of the PAU and new IMH in 20% and con-
version to aortic dissection in 10%.3 All patients in the
series went on to require operative repair. In contem-
porary series, most patients with symptomatic PAU
without rupture have been treated with open or
endovascular repair with acceptable results (see PAUs that are asymptomatic and without high-risk
Section 7.6.3, “PAU: Open Surgical Repair Versus features have a low risk of progression (3.6% and
Endovascular Repair”). 3,7-9
6.5% at 5 and 10 years after diagnosis, respectively).10
2. Asymptomatic isolated PAU with large diameter or Maximum depth and diameter of the PAU can be
depth, significant growth on surveillance imaging, or used to determine lesions that would be considered
other high-risk features (Table 31), are associated with high risk and may be considered for intervention
disease progression.1,7 In contrast, incidental aortic (Figure 22).4
7.6.3. PAU Open Surgical Repair Versus Endovascular Repair
Recommendations for PAU Open Surgical Repair Versus Endovascular Repair
1. In patients who require repair of a PAU in the ascending aorta or proximal aortic arch (zones 0-1), open
1 C-LD surgical repair is recommended.
2. In patients who require repair of a PAU in the distal aortic arch (zones 2-3), descending thoracic aorta, or
2a C-LD abdominal aorta, either open surgical repair 1-3 or endovascular repair is reasonable, based on anatomy
and medical comorbidities.4-6
Synopsis proximal arch, with acceptable morbidity and mortality

Operative repair of PAUs includes both open and rates compared with medical therapy.12,13
endovascular treatment. Historically, most PAUs were
treated with open aortic replacement, although more
contemporary series have reported good technical success 1. Results of open surgical repair of the ascending aorta
and short- and midterm outcomes after endovascular and proximal arch can be reasonably applied to PAU.
repair in the descending and infrarenal aorta.4-7 Cases have been reported in which ascending aortic
Comparative data are limited about the best treatment stenting has been performed with surgeon-modified
approach for a PAU but, in general, the approach depends stent-grafts or off-label use of commercially available
on the location of the PAU, the patient’s aortic and branch devices, but currently there is no FDA-approved device
vessel anatomy, associated pathology, and patient for endovascular repair of the ascending aorta or
comorbidities (because these patients tend to be older proximal arch.
and have significant atherosclerosis).4 Procedure-related 2. The risk of procedure-related and in-hospital death is
and in-hospital death are lower for patients treated with lower for endovascular compared with open repair of
an endovascular approach, although available data are PAU in the descending thoracic and abdominal aorta,
based on small studies with a high risk of treatment bias.4 although longer-term data are similar for both opera-
Midterm outcomes after endovascular repair of PAU have tive approaches.4
4,7
shown a 4% to 8% risk of endoleak and a 5% risk of new
PAU formation.7 One-year mortality rates for patients
7.7. Traumatic Aortic Injury
treated with endovascular versus open repair are similar.7
7.7.1. Initial Management of Blunt Traumatic Thoracic Aortic
Results of open surgical repair in patients with
8-11 Injury (BTTAI)
ascending aortic PAU are limited to small case series.
Despite this, open repair remains the gold standard for 7.7.1.1. Initial Management of BTTAI in the Emergency
treating AAS that involve the ascending aorta and Department
Recommendations for Initial Management of BTTAI in the Emergency Department
1. In patients with BTTAI, management and treatment at a trauma center with the facilities and expertise to
1 C-EO treat aortic pathology is recommended.
2. In patients with BTTAI, anti-impulse therapy to reduce the risk of injury extension and rupture should be
1 C-LD implemented, except in patients with hypotension or hypovolemic shock. 1,2

BTTAI, although rare, is the second-most common
1. Patients with BTTAI are at elevated risk of aortic-
cause of death in trauma patients; it results from high
related and overall mortality. Because of the acuity of
deceleration forces and is often associated with concom-
injury and severity of concomitant polytrauma,
itant injuries. In the ACS National Trauma Databank, the
expertise in aortic imaging and treatment at the treat-
diagnosis of BTTAI increased 196.8% from 2003 to 2013,
ing facility is paramount to improve outcomes.
likely attributable to more sensitive imaging. 3 The mor-
Further, most patients will benefit from care at a level 1
tality rate of patients with BTTAI who were treated in the
trauma center with multidisciplinary expertise in
emergency department was w19%.4,5 Initial management
treating concomitant injuries, although the risk of
of polytrauma at trauma centers follows Advanced
delayed treatment because of transport time must be
Trauma Life Support protocols. However, for patients
weighed against the benefits of immediate treatment.
with BTTAI, special attention to BP and heart rate is
2. Although no randomized trials exist, historical litera-
warranted because of their effects on injury extension
ture shows that aortic rupture occurs in w12% of pa-
and rupture.
tients with BTTAI who were awaiting repair without
In stable patients, the 2011 Society for Vascular
medical management; small studies using protocols of
Surgery clinical practice guidelines6 suggested urgent
beta blockers as first-line therapy have reported rates
(<24 h) repair barring other serious concomitant
of 0% rupture while awaiting repair. 1,2 In the acute
nonaortic injuries or immediately after treatment of
trauma setting, hypovolemia may result in permissive
other injuries. Optimal timing of intervention, how-
hypotension, obviating the need for administering
ever, remains unclear. In a recent study from the
anti-impulse medications (typically intravenous beta
National Trauma Data Bank, early (<24 h) repair had
blockers with or without supplemental intravenous
increased odds of death (adjusted OR, 2.39; 95% CI,
vasodilators [eg, nicardipine, clevidipine, sodium
1.01–5.67; P¼0.047).7 A multicenter study showed
nitroprusside]) to decrease aortic wall stress.
worse adjusted mortality rate with early repair overall
Conversely, permissive hypotension may not be toler-
(adjusted OR, 7.78; 95% CI, 1.69–35.70; adjusted
ated with other concomitant injuries, in which
P¼0.008) although, in subgroup analysis, mortality
adequate end-organ perfusion requires higher BPs.
rate differences only trended toward favoring delayed
repair (P>0.05).8 7.7.1.2. Approach to the Initial Management of BTTAI
Recommendations for Approach to the Initial Management of BTTAI
1. In patients with grade 1 BTTAI (Figure 23), nonoperative management and follow-up imaging are
1 C-LD recommended. 1,2
2. In patients with grade 3 to 4 BTTAI (Figure 23) and nonprohibitive comorbidities or injuries, aortic
1 C-LD intervention is recommended. 1,3
3. In patients with grade 2 BTTAI (Figure 23) and with high-risk imaging features (Table 32), aortic inter-
2a C-LD vention is reasonable. 3,4
4. In patients with grade 2 BTTAI (Figure 23) and without high-risk imaging features (Table 32), nonoper-
2b C-LD ative management and follow-up surveillance imaging may be reasonable. 3,4
Synopsis segments that may be involved include the proximal

The most common site of BTTAI is the aortic isthmus, ascending aorta (8%–27%), aortic arch (8%–18%), and
because of its site as transition from the unfixed aortic distal descending thoracic aorta (11%–21%). The most
arch to the fixed descending thoracic aorta and the rela- widely used grading scale is that proposed by Estrera et al
tively lesser tensile strength of this region. Other and endorsed by the SVS clinical practice guideline
F I G U R E 2 3 Classification System for BTTAIs
Aortic injuries are classified according to severity, based on the findings of diagnostic imaging. Grade 1, intimal tear. intimal flap, or both. Grade 2, intramural hematoma.
Grade 3, aortic wall disruption with pseudoaneurysm. Grade 4, aortic wall disruption with free rupture. BTTAI indicates blunt traumatic thoracic aortic injury. Adapted
from Azizzadeh et al,2 Copyright 2009, with permission from Elsevier, Inc. and the Society for Vascular Surgery.
(Figure 23).1,2 In Estrera’s original paper, all patients with tors such as the patient’s stability, concomitant
grade 1 injuries were managed medically and had a 0% injuries, and potential imaging characteristics that may
mortality rate.2 Current SVS guidelines recommend predict aortic stability. Grade 1 BTTAIs are likely to
expectant management of grade 1 injuries and repair of all resolve and are associated with extremely low aortic-
other grades.1 Trauma studies have found that 32% of related death. Medical management and follow-up
BTTAIs are managed nonoperatively,3 with an associated imaging to ensure resolution is appropriate.1,2
5
mortality rate of 25%. Overall mortality rate was signifi- 2. Grade 3 and 4 BTTAIs are at high risk of progression
cantly higher in nonoperatively managed patients (35.0% and rupture and should be treated in an urgent
versus 11.2%; P<0.001), while aortic-related mortality rate manner. In grade 3 injuries, nonoperative manage-
was similar (9.8% versus 5.0%; P¼0.119).3 ment was an independent predictor of all-cause death
(OR, 29.65; 95% CI, 5.62–15.649; P<0.0001), and im-
Recommendation-Specific Supportive Text: Management aging characteristics did not predict aortic-related
death.4
1. The decision for nonoperative versus operative man-
3. Although injury grade was an independent predictor of
agement of BTTAI includes complex and dynamic fac-
aortic-related death, outcomes of grade 1 and 2 injuries
were similar between nonoperative management and
TEVAR, including in-hospital and aortic-related death
(P>0.05). 3 A high-volume center reported no differ-
ences in mortality rates or aortic-related mortality
TABLE 32 High-Risk Imaging Features of BTTAI
rates between nonoperative and operative manage-
n Posterior mediastinal hematoma >10 mm 8
ment of grade 1 and 2 injuries. 4
n Lesion to normal aortic diameter ratio >1.48
n Mediastinal hematoma causing mass effect6
4. Findings of secondary signs of injury and multiple
n Pseudocoarctation of the aorta6 secondary signs are more common in patients with
n Large left hemothorax6 higher-grade of aortic injury and may prompt stronger
n Ascending aortic, aortic arch, or great vessel involvement 9 consideration for operative intervention.6 The pres-
n Aortic arch hematoma7
ence of aortic arch hematoma of >15 mm in thickness
BTTAI indicates blunt traumatic thoracic aortic injury. was predictive of death.7
7.7.1.3. Endovascular Versus Open Surgical Repair
Recommendation for Endovascular Versus Open Surgical Repair

1. In patients with BTTAI who meet indications for repair and with appropriate anatomy, TEVAR is
1 B-NR recommended over open repair.1-3

Endovascular therapy for BTTAI has become the pre-
1. Compared with open repair, endovascular treatment of
dominant approach. From 2007 to 2015, rates of open
BTTAI is associated with improved procedural and 30-
repair decreased from 7.5% to 1.9%, while rates of TEVAR
2 day mortality rates, as well as postoperative complica-
increased from 12.1% to 25.7%.
tions, including SCI and acute kidney injury. 1-3,6 . In a
No randomized trials for open versus endovascular
meta-analysis of 17 retrospective studies, TEVAR was
management have been conducted.4 Rather, trauma
associated with lower procedural and 30-day mortality
registry data and meta-analyses have shown that, in
rates (OR, 0.31 and 0.44, respectively) and post-
patients with suitable anatomy, TEVAR offers superior
operative paraplegia (OR, 0.32).1 Murad et al showed
30-day mortality rates and lower rates of SCI and acute
similar reductions in mortality (relative risk, 0.61) and
kidney injury. Concomitant injuries may prompt
SCI (relative risk, 0.34) in 139 studies encompassing
concern over procedural use of heparin, and the use of
7,768 patients.3 Studies using the National Trauma Data
periprocedural heparin should be balanced against the
Bank, a multicenter registry of trauma centers, also
overall bleeding risk for each patient. In a small study
have identified significantly improved mortality rates,
of TEVAR in patients with predominantly grade 3
shorter ICU and shorter hospital stay, and lower rates of
BTTAI, there were no differences in bleeding, throm-
acute kidney injury and acute respiratory distress
boembolism, or mortality rates between use of full
syndrome 2,6 for TEVAR compared with open repair.
heparin, low-dose heparin, and no heparin, although
patients who received full heparin underwent repair at
7.7.2. Initial Management of Blunt Traumatic Abdominal Aortic
a time interval 3 times longer than did those who
Injury (BAAI)
received no heparin.5
Recommendations for Initial Management of BAAI

1. In patients with grade 1 to 2 BAAI (Table 33) without malperfusion, anti-impulse therapy, if clinically
1 C-LD tolerated, and repeat imaging within 24 to 48 hours of the initial scan is recommended to reduce risk of
injury progression. 1
2. In patients with grade 4 BAAI (Table 33), repair should be performed to address life-threatening aortic
1 C-LD injury. 2-4
3. In patients with grade 2 BAAI (Table 33) and associated malperfusion, it is reasonable to consider repair. 1
2a C-LD
4. In patients with BAAI, treatment with either endovascular or open repair is reasonable and depends on
2a C-LD degree of injury, aortic anatomy, and the patient’s overall clinical status. 1-4
5. In patients with grade 3 BAAI (Table 33), it may be reasonable to consider repair to reduce risk of
2b C-LD progression to life-threatening injury.5
6. In patients with BAAI, the usefulness of routine application of resuscitative endovascular balloon oc-
3: Harm B-NR clusion of the aorta (REBOA) for hemorrhage control is unclear and, in some cases, may cause harm.6-8
Synopsis emergency department (eg, antero-lateral thoracotomy

BAAI represents a rare traumatic entity, occurring with aortic cross-clamping) or operating room. Whether
in <1% of patients with blunt trauma. Patients with BAAI open or endovascular means are used for BAAI repair will
often have concomitant injuries such as rib fractures, depend on patient’s clinical status, hospital resources,
abdominal visceral injury, and cardiac complications that and practitioner experience. Additionally, Shalhub et al1
will affect treatment decisions. Similar to BTTAI, propose using aortic injury zone categorization when
abdominal aortic injuries are graded based on aortic considering options for repair, which differ from trau-
contour defects, and this grading can be used to provide a matic abdominal zones of injury (Figure 24). Specifically,
framework for treatment and determination of risk of in their multicenter experience, some zone 2 and 3 in-
major morbidity and death from injuries (Table 33). juries could be managed endovascularly while no zone 2
Because BAAI is rare and symptoms are wide ranging, injuries were managed this way. Lastly, data on the use of
patients should be managed on an individual basis. In REBOA for hemorrhage below the diaphragm, not per-
general, patients with grade 1 aortic injuries can likely be formed in the operating theater, and without fluoroscopic
managed with antihypertensive therapy, beta blockade, guidance are mixed, with few data showing survival
and antiplatelet therapy, if not contraindicated, with benefit and some trauma registry data showing harm.
repeat scan at 24 to 48 hours. Grade 2 injuries can simi-
larly be managed nonoperatively but may progress to
include end-organ vessel thrombosis or rupture. Grade 3 1. Because BAAI is very rare, in an effort to provide
injuries may benefit from endovascular treatments if clinical evidence for the management of BAAI, Shalhub
anatomically amenable. Grade 4 injuries are more likely et al1 aggregated data from 12 trauma centers. In the
to present with refractory hypotension, warranting rapid authors’ experience in treating 113 patients with BAAI,
control of hemorrhage, which may be done in the most of those with grade 1 and 2 injuries were
F I G U R E 2 4 Abdominal Aortic Zones of Injury for Surgical Approaches and Abdominal Zones of Injury Based on Trauma Classification
(A) The abdominal aortic zones of injury described by Shalhub et al.1 (B) The abdominal zones of injury traditionally described in trauma. The abdominal aortic zones of
injury may help in prognostication and deciding whether an endovascular or open repair is feasible. Shalhub et al1 found that the mortality rate was highest in zone 2
(see panel A) grade 4 aortic injuries (Table 33). Moreover, no zone 2 aortic injuries identified in a multicenter experience were managed by endovascular means. Panel A,
adapted from Shalhub et al.1 Copyright 2014, with permission from Wolters Kluwer Health, Inc.
those initially managed conservatively, 3 eventually

TABLE 33 Descriptions of Blunt Aortic Injury Grades
progressed to having ischemic symptoms warranting
Injury consideration of repair, with 1 patient who refused
Grade Descriptions
repair who died of sepsis from limb ischemia, another
1 Minor intimal tear, intimal defect, or thrombus (#10 mm)
who died intraoperatively, and a third who success-
2 Large intimal flap, intimal defect, or thrombus ($10 mm in
length or width) fully underwent hybrid endovascular and open repair.
3 Pseudoaneurysm 4. Both endovascular and open approaches have been
4 Aortic rupture
described for BAAI,1-4 and analyses of large trauma
databases reveal no significant differences in mortality
In their descriptions of management of BAAIs, Shalhub et al1,2 use an aortic injury
grading system described by Starnes et al13. Instead of using IMH to define grade 2
rates between the two. Anatomical considerations,
injuries, as did Azizzadeh et al,14 Starnes et al13 define grade 2 injuries based on a higher patient clinical status and comorbid injuries, and
degree of intimal injury, defect, thrombus, or all of them to match radiographic findings
that they deemed to be less ambiguous.
practitioner experience will influence the choice of
BAAI indicates blunt traumatic abdominal aortic injury; and IMH, intramural approach. Shalhub et al 1 found that aortic zone 2 and 3
hematoma.
injuries appeared to be more amenable to endovascular
approaches, while most grade 4 injuries were treated
successfully managed nonoperatively with anti- with open surgery. 1,2 Currently, no FDA-approved de-
impulse therapy and repeat CTA imaging. Most of vices are available specifically for treating trauma in
these injuries did not show progression and did not the abdominal aorta; consequently, clinical judgment
require in-hospital intervention. However, some pa- and experience are paramount in choosing an endo-
tients will develop angiographic progression of lesions vascular solution.
or develop symptoms from vessel occlusion, aneu- 5. Pseudoaneurysm repair is often performed to prevent
rysmal degeneration, or pseudoaneurysm formation. progression to uncontrolled aortic rupture, although
Such progression should prompt consideration of data on characteristics associated with progression are
treatment to prevent further progression to symp- scarce. In their multicenter study of BAAI, Shalhub
tomatic or life-threatening disease. et al1 found that only 30% of pseudoaneurysms were
2. Patients with grade 4 injuries are more likely to present managed nonoperatively, and failure of nonoperative
with hypotension and aortic transection as well as management occurred in 3 of these patients.
visceral vessel avulsion.2-4 In a single-center experi- 6. REBOA has reemerged over the past 10 years as a form
ence, all 8 patients with grade 4 injuries experienced of rapid hemorrhage control in trauma. Many health
cardiac arrest in the emergency department or oper- care centers have shown the feasibility of trauma sur-
ating room. Although all 8 patients survived to reach geon or emergency physician placement of endovas-
the operating room and 7 survived the repair, all died cular balloons for hemorrhage control. 6,9,10 with a few
within days of injury. In multicenter experience, the studies showing significant improvement in SBP after
mortality rate for grade 4 injuries was 83%. Most placement11 and survival benefit compared with those
deaths from BAAI were within the first 24 hours of who were not treated with REBOA 12 or those treated
presentation and attributable to cardiac arrest from with open methods of hemorrhage control.8 However,
hemorrhagic shock. propensity-matched studies using large trauma data-
3. Patients may present with grade 2 injuries without bases showed increased mortality rate and risk of
evidence of malperfusion and thus be managed non- complications, such as acute kidney injury, amputa-
operatively. However, for patients who present with or tion, or both, with use of REBOA. 6-8 There are clinical
progress to organ or limb malperfusion, endovascular scenarios in which REBOA is contraindicated. Accord-
or open repair may be needed to reduce morbidity and ing to the current US Army Joint Trauma System clin-
mortality rates. In their multicenter experience, Shal- ical practice guidelines, REBOA is contraindicated in
hub et al 1 found that of the 38 patients who present those with pericardial tamponade and major thoracic
with grade 2 injuries, 45% were initially managed hemorrhage. Relative contraindications to REBOA use
nonoperatively, 34% were treated with open repair, include cardiac arrest or shock caused by penetrating
and 21% were treated with endovascular repair. Of chest trauma.
7.7.3. Long-Term Management and Surveillance After Blunt

Traumatic Aortic Injury (BTAI)
Recommendations for Long-Term Management and Surveillance After BTAI
1. In patients with BTAI who have undergone aortic repair, surveillance imaging at intervals appropriate for
2a C-LD the repair approach and location (see Section 7.8, “Long-Term Management and Surveillance Imaging
Following AAS”) is reasonable.1-4
2. In patients with BTAI who have not undergone repair, surveillance imaging with a CT at 1 month,
2b C-LD 6 months, and 12 months after the diagnosis and, if stable, at appropriate intervals thereafter
(depending on the type and extent of the injury), may be reasonable. 5

In-hospital and midterm outcomes after open and
1. In-hospital data suggest that endograft malposition
endovascular repair for BTAI are good for patients who
(3%) and endoleak (2%) may occur in some patients
survive to hospital discharge. 2-4 However, long-term data
immediately after endovascular repair,1 but midterm
are limited that report outcomes after open or endovas-
data after open or endovascular repair of BTAIs suggest
cular surgical repair for blunt aortic injury. The SVS
a low incidence of endoleak, stent migration, or rein-
clinical practice guidelines for traumatic thoracic aortic
tervention after a mean of 52 to 60 months. 2-4 Long-
injury suggest that follow-up after TEVAR could be
term data for outcomes of open or endovascular
decreased to every 2 to 5 years in the absence of abnor-
repair of BTAI are lacking.
malities on follow-up imaging (ie, stent graft migration,
2. Among patients with blunt traumatic injury who are
endoleak) or could follow-up standard postoperative im-
managed nonoperatively, injury progression occurred
aging surveillance paradigms.6 No published guidelines
in 7.6% of patients, and injury healing or improvement
are available for postoperative surveillance after open or
was observed in 34% of patients after a range of 1 day to
endovascular abdominal aortic repair for blunt aortic
118 months of follow-up. 5 Injury progression, inter-
injury.
vention, or both occur in 0.68% of patients with grade 1
Long-term data about outcomes of blunt aortic injuries
to 2 BTAI.5 Long-term data for outcomes of blunt aortic
managed nonoperatively are limited. A recent systemic
injuries managed nonoperatively are lacking.
review of nonoperative management of blunt thoracic
aortic injuries showed low aortic-related event rates but
injury progression in 7.6% of patients on surveillance 7.8. Long-Term Management and Surveillance Imaging
imaging (follow-up, 1 day to 118 months).5 In published After AAS
series of blunt aortic injury, patients with disease pro- 7.8.1. Long-Term Surveillance Imaging After Aortic Dissection
gression on repeat imaging all undergo repair.4,5 and IMH
Recommendations for Long-Term Surveillance Imaging After Aortic Dissection and IMH
1. In patients who have had an acute aortic dissection and IMH treated with either open or endovascular
1 B-NR aortic repair and have residual aortic disease, surveillance imaging with a CT (or MRI) is recommended
after 1 month, 6 months, and 12 months and then, if stable, annually thereafter. 1-6
2. In patients who have had an acute aortic dissection and IMH that was managed with medical therapy
1 B-NR alone, surveillance imaging with a CT (or MRI) is recommended after 1 month, 6 months, and 12 months
and then, if stable, annually thereafter. 7
Synopsis surveillance imaging is essential to detect midterm and

Survival after an acute aortic dissection and IMH does late aortic growth. 7
not guarantee freedom from subsequent aortic events 2. After surgical replacement of the ascending thoracic
because of residual aortic dissection and risk of aneurysm aorta in acute type A aortic dissection, patients
formation. Ten-year survival after repair of acute type A remain at risk for progressive enlargement of unre-
aortic dissection is approximately 60% to 65%.1,8 Risk of paired segments of residual dissected aorta, as well
reoperation is increased for the aortic valve, the aortic as potential growth of nondissected native aortic
root, and the distal aorta,1,8,9 with an aortic root reoper- segments because of underlying medial degeneration.
ation rate of approximately 15% at 15 years.9,10 The Consequently, repeat intervention on the aortic root,
growth rate of the distal aorta is w1 mm/y, and the risk of arch, or thoracoabdominal aorta may become neces-
distal aortic reoperation ranges from 10% to 16% at 10 sary. For acute type B aortic dissection, 1,2 TEVAR may
1,8,9
years. Although the use of TEVAR provides protection leave a patent false lumen, which can lead to aneu-
from early aortic-related death11 in acute type B aortic rysm growth, and can be complicated by early
dissection, reintervention rates after TEVAR for can range endoleaks in up to 35% of patients and late endoleaks
from 27% to 39% at midterm follow-up.11,12 Surveillance in 13% of patients. 5 Careful follow-up is needed to
imaging after thoracic aneurysm repair is critical to monitor for progression of disease in both dissected
monitor for progression of residual aortic disease and the and nondissected aorta. In addition to using cross-
potential need for reintervention. sectional imaging for most of the aorta, TTE can be
helpful in monitoring aortic root anatomy and aortic
valve function over time.
1. Although patients with uncomplicated type B aortic
dissection who are managed medically have a favorable 7.8.2. Long-Term Management After Acute Aortic Dissection
early prognosis, delayed aortic expansion occurs in and IMH
20% to 50% of patients over 4 years,7 so regular
Recommendation for Long-Term Management After Acute Aortic Dissection and IMH
1. In patients with a previous acute aortic dissection and IMH, whether initially treated medically or with
1 B-NR intervention, who have chronic residual TAD and an aneurysm with a total aortic diameter of ‡5.5 cm,
elective thoracic aortic repair is recommended.1-4
Synopsis indications for reoperation are aneurysms of the thoracic

Despite the outcomes reported for surgical repair of aorta, aortic anastomotic pseudoaneurysms, progressive
acute aortic dissection and IMH, a risk of ongoing growth AR, or graft infection.1 Operative mortality rate of
is possible in the residually dissected as well as non- elective repair is <10%.1-4 After TEAVR, false-
dissected thoracic aortic segments. When surveillance lumen thrombosis can occur in 62% of extent 3B
imaging detects progression of residual aortic disease after dissection and 91% of extent 3A dissection cases. Rein-
successful treatment of acute aortic dissection and IMH, tervention rates after TEVAR range from 15% to 26% at 5
there may be a potential need for aortic reintervention. years and are dependent on the extent of aortic
dissection.4
1. Reoperation after acute type A aortic dissection repair is 7.8.3. Long-Term Management and Surveillance for PAUs
associated with low rates of complication. The primary
Recommendations for Long-Term Management and Surveillance for PAUs
1. In patients with a PAU who have undergone aortic repair, surveillance imaging at intervals appropriate for the
2a C-LD repair approach and location (see Section 6.5.6, “Surveillance After Aneurysm Repair”) is reasonable.1-3
2. In patients with a PAU that is being managed medically, surveillance imaging with a CT is reasonable at 1
2a C-LD month after the diagnosis and, if stable, every 6 months for 2 years, and then at appropriate intervals
thereafter (depending on patient age and PAU characteristics).1,4
Synopsis postoperatively. 1 Freedom from cumulative complica-

For patients who undergo repair of a PAU, clinical tions and interventions is 86% at 12 months, 79% at 24
failure (defined as endoleak, disease progression, graft months, and 71% at 36 months postoperatively.2 After
occlusion, repeat aortic intervention, or procedure or 18 months of follow-up, new PAUs are observed in
aortic-related death) by 1 year after endovascular and approximately 5% of patients who have undergone
open repair occur in 8.6% and 8.7%, respectively. 1 No repair of a different PAU.3
long-term data exist for outcomes after repair of PAU, but 2. In a series of 109 patients with acute PAUs, 28% suf-
aortic-related complication rates after intervention are fered from an aortic-related adverse event by 30 days
likely similar to those for TAA. of follow-up. 4 Based on a systematic review of 184
For patients with PAUs who are managed non- patients with either thoracic or aortic PAU, 30% had
operatively, the risk of disease progression is signifi- radiographic evidence of disease progression on
cant. 1,5,6 Disease progression occurs more frequently in midterm follow-up.1 For patients with abdominal PAU,
patients presenting with symptomatic versus asymptom- 23% have disease progression on CTA by 8 months of
atic PAUs 7 but is >15% for both. 7,8 Among patients with a follow-up, although the risk is higher in symptomatic
PAU who have progression of disease on surveillance (43%) versus asymptomatic (17%) patients. 7
imaging, 73% will show continued worsening on subse-
quent imaging, and 46% will have progression to frank
dissection after a mean of 12 months.
8. PREGNANCY IN PATIENTS WITH AORTOPATHY
1. After open or endovascular repair of a PAU, there is a 8.1. Counseling and Management of Aortic Disease in
9% risk of clinical failure by 12 months Pregnancy and Postpartum
Recommendations for Counseling and Management of Aortic Disease in Pregnancy and Postpartum
Prepregnancy
1. In patients with genetic aortopathies attributable to syndromic (Marfan syndrome, Loeys-Dietz syn-
1 C-LD drome, vascular Ehlers-Danlos syndrome) and nsHTAD and who are contemplating pregnancy, genetic
counseling before pregnancy to discuss the heritable nature of their condition is recommended. 1-4
2. In patients with syndromic and nsHTAD, Turner syndrome, BAV with aortic dilation, and other aortopathy
1 C-LD conditions, aortic imaging (with TTE, MRI or CT, or both as appropriate) before pregnancy is recom-
mended to determine aortic diameters. 1-3,5-13
3. In patients with syndromic and nsHTAD, Turner syndrome, BAV with aortic dilation, and other aortopathy
1 C-LD conditions, who are contemplating pregnancy, counseling about the risks of aortic dissection related to
pregnancy is recommended. 2-5,10,12,14
During Pregnancy
4. In patients with aortic aneurysms, or at increased risk of aortic dissection, or both, it is recommended that
2a C-EO pregnancy be managed by a multidisciplinary team including a maternal fetal medicine specialist and
cardiologist, and, if logistically feasible, that delivery be planned in a hospital where the capability for
emergency aortic repair is available.
5. In patients with aortopathies who are pregnant, guideline-directed treatment of hypertension is

1 C-LD recommended. 6,15,17
6. In patients with syndromic and nsHTAD, beta-blocker therapy during pregnancy and postpartum is
1 C-EO recommended, unless contraindicated.
(Continued)
7. In pregnant patients with an aortopathic condition or a dilated aortic root or ascending aorta, surveillance
1 C-LD TTE to monitor aortic diameters and aortic valve function is recommended each trimester and again
several weeks postpartum, although imaging may be more frequent depending on aortic diameter, aortic
growth rate, and underlying condition. 7-9,17,18
8. In pregnant patients with aortic disease who require surveillance imaging of the aortic arch, descending,
1 C-LD abdominal aorta, or all 3, MRI without gadolinium is recommended over CT to avoid radiation exposure to
the fetus. 19,20
Synopsis 2. Women with aortopathic conditions are at risk for

Pregnancy leads to hemodynamic and hormonal aortic dilation and aortic dissection related to preg-
changes and is a risk factor for aortic dissection in women nancy. 6,7,14,22 Evaluation of the aortic root, ascending
21
with aortopathy. Aortic dissection may occur aorta, or both by echocardiogram before pregnancy in
throughout pregnancy or several weeks postpartum, with women with aortopathy is important for prepreg-
most in the third trimester or up to 12 weeks’ post- nancy counseling and management during preg-
partum.21 Women with aortopathy, including Marfan nancy. 1-3,5-7,9,11,13 In conditions that associate with
6,7,13,14,18,19,22 2,22,23
syndrome, Loeys-Dietz syndrome, aortic disease distal to the ascending aorta, prepreg-
vascular Ehlers-Danlos syndrome,3,24 nsHTAD,25,26 nancy MRI or CT is performed to evaluate for aortic
Turner syndrome, 12,27 and BAV with aneurysm 21,28 are at disease. 2,5,9,14
risk of pregnancy-related aortic dissection. Type A aortic 3. The risk of type A aortic dissection in pregnancy relates
dissection in pregnancy associates with aortic dilation, to the aortopathy condition and aortic diameter, but
but type B aortic dissection may occur without aortic type B aortic dissection may occur without significant
dilation.6,13,22 aortic dilation.6,22 Most dissections related to preg-
Before pregnancy, women with or at risk for aortopathy nancy occur in the third trimester and in the first 12
undergo TTE (and MRI or CT, as appropriate) and are weeks’ postpartum.21 Awareness of the signs and
counseled about risks of aortic dissection informed by symptoms of acute aortic dissection among stake-
specific circumstances. Aortic surveillance imaging holders may improve diagnosis and outcomes. In
throughout pregnancy and several weeks postpartum is Marfan syndrome, type A aortic dissection risk is very
performed to monitor aortic size. 9 low when aortic diameters are <4.0 cm and are much
In women at low risk, vaginal delivery is performed higher at diameters >4.5 cm. In series of women with
with efforts to lessen hemodynamic stress and shorten TGFBR1 or TGFBR2 pathogenic variants, aortic dissec-
the second stage of labor.9,14 Women at increased risk of tion was reported in 0% to 19% of pregnancies.21 Rapid
aortic complications typically undergo cesarean aortic growth in pregnancy is reported in Loeys-Dietz
delivery. 9,14 syndrome. 2 Limited data are available on pregnancy
In women with aortopathy, prepregnancy genetic and SMAD3, TGFB2, or TGFB3 pathogenic variants. 31,32
counseling, aortic imaging, discussion about aortic Maternal mortality rates in vascular Ehlers-Danlos
dissection risk, and shared decision-making are syndrome have ranged from 4% to 25%. 3 Among 283
9
necessary. women with vascular Ehlers-Danlos syndrome with 616
delivered pregnancies, 30 women died, with a
pregnancy-related death rate of 4.9%.3 Pregnancy has
1. HTAD encompasses conditions in which aortic disease typically been avoided in women with vascular Ehlers-
has an underlying genetic trigger or familial occur- Danlos syndrome. 9 The decision to proceed with
29,30
rence. Marfan syndrome, Loeys-Dietz syndrome, pregnancy in vascular Ehlers-Danlos syndrome is
vascular Ehlers-Danlos syndrome, and nsHTAD are complex and, for some women with specific genetic
autosomal dominant conditions with an inheritance variants, null mutations, and normal vascular imaging,
risk of 50%.9,30 BAV may also be familial. Prepregnancy the risk may be lower, and shared decision-making is
counseling by a genetic counselor, medical geneticist, required.3 Aortic dissection at small aortic diameters
or both or aortopathy specialist is recommended to has been reported in some patients with
discuss the heritability of these conditions and to nsHTAD.25,26,33 Aortic dissection related to BAV is rare
identify at risk relatives and to discuss pregnancy and, when reported, associates with aneurysmal
concerns.9,30 dilation. 14,21,28
4. For women with aortopathic conditions, multidisci- Cardiac disease), there was no significant difference in
plinary evaluation before and throughout pregnancy birth weight in women treated with a beta blocker
can evaluate and manage BP, aortic diameter, and compared with untreated women (2,960 g [2,358–3,390
monitor pregnancy for complications. Delivery in a g] versus 3,270 g [2,750–3,570 g]); P¼0.25).14 Because
setting in which cardiothoracic surgery is available for aortic dissection may occur postpartum, beta-blocker
urgent management of aortic dissection allows rapid therapy is continued for at least several weeks after
treatment for this complication. 25 Educating women delivery and indefinitely for those with indications for
with aortopathic conditions and their physicians about long-term use.
the signs and symptoms of acute aortic dissection may 7. Pregnancy-associated increases in maternal blood
allow earlier diagnosis and improve outcomes.12,21,25 volume, heart rate, stroke volume and cardiac output,
5. Hypertension is a risk factor for aortic dissection in and neurohormonal activation begin in the first
pregnancy.6 For appropriate patients with or without trimester and peak in the third trimester and peri-
hypertension, beta blockers are used throughout partum period.9 In women with aortopathic condi-
pregnancy and postpartum, recognizing that fetal tions, the aorta may dilate during pregnancy, 7 and
13,15
growth may be impaired. Labetalol is suggested as significant dilation is a risk factor for ROPAC. 8,14 Aortic
the beta blocker of choice for use in pregnant women imaging frequency during pregnancy is variable and is
with hypertension. 35 Other agents may be required as performed every trimester but may be performed more
suggested by international guidelines.16,34 ARBs and frequently depending on individual factors, including
ACEIs are contraindicated during pregnancy because of the specific aortopathic condition, aortic diameter, and
teratogenicity. Calcium channel blockers are generally rate of aortic growth. 3,5,9,10,12-14,25 Evaluation of the
avoided, when possible, in Marfan syndrome based on aorta several weeks postpartum to determine aortic
limited information and concerns raised from mouse diameter is performed to evaluate for aortic dilation. 9
34,35
models. 8. In patients with aortopathy that involves the aortic
6. Beta-blocker therapy has been shown to lessen aortic arch, descending or abdominal aorta or branches, or all
growth rates in Marfan syndrome and is recommended of them, cross-sectional imaging identifies aortic
to lessen hemodynamic aortic stress in Marfan syn- anatomy and diameters. MRI without gadolinium
drome and related conditions.4,5,13 In the absence of contrast is low-risk during pregnancy and is preferred
controlled trials, beta blockers are used in other aor- over CT for elective imaging to avoid the risks of
topathic conditions, and continuation of such therapy ionizing radiation exposure to the developing
during pregnancy is recommended unless contra- fetus.9,19,20 A TEE can be performed during pregnancy,
indicated. 2,5,9 Shard decision-making is required, un- if required, to evaluate the descending aorta.
derstanding that fetal growth and weight may be
impaired when beta blockers are used in pregnancy. 15
8.2. Delivery in Pregnant Patients With Aortopathy
However, in ROPAC (Registry Of Pregnancy And
Recommendations for Delivery in Pregnant Patients With Aortopathy
1. In pregnant patients with a history of chronic aortic dissection, cesarean delivery is recommended.
1 C-EO
2. In pregnant patients with an aortopathy and an aortic diameter of <4.0 cm, vaginal delivery (when
1 C-EO otherwise appropriate) is recommended.
3. In pregnant patients with a diameter of the aortic root, ascending aorta, or both, of ‡4.5 cm, cesarean
2a C-EO delivery is reasonable.
4. In pregnant patients with a diameter of the aortic root, ascending aorta, or both, of 4.0 cm to 4.5 cm,
2b C-EO vaginal delivery with regional anesthesia, expedited second stage, and assisted delivery may be
reasonable.
5. In pregnant patients with syndromic and nsHTAD, and a diameter of the aortic root, ascending aorta, or
2b C-EO both, of 4.0 cm to 4.5 cm, cesarean delivery may be considered.
Synopsis 2. Type A and type B aortic dissection related to preg-

The risk of type A aortic dissection related to pregnancy nancy may occur in Marfan syndrome.1,2,6 Women
in Marfan syndrome is related to aortic root diameter, with with aortic root dilation >4.0 cm and, especially >4.5
a low risk (w1%) of aortic dissection at an aortic cm, are at increased risk of type A aortic dissection
diameter <4.0 cm and much greater risk at aortic diameters during pregnancy and postpartum.1,3 Aortic dissec-
1-3
>4.5 cm. Progressive aortic dilation and hypertension tion has been reported to be low risk in small series
also determine dissection risk.4-6 Complex and shared of women with aortic diameters between 4.0 cm and
decision-making is required when the aorta is between 4.0 4.5 cm,2,7,8 but aortic dissection related to pregnancy
cm and 4.5 cm in diameter, recognizing that although some at this diameter may occur.1 Type B aortic dissection
2,7,8
series report low risk, aortic dissection related to related to pregnancy may occur without significant
pregnancy at this diameter may occur.1 Modified World aortic dilation and after previous aortic root
Health Organization classification on cardiovascular risk replacement. 1,11
places women with Marfan syndrome and moderate aortic 3. In the absence of controlled trials, cesarean delivery is
dilation of 4.0 cm to 4.5 cm in modified World Health Or- often performed in women with Marfan syndrome and
ganization class III and those with aortic diameter >4.5 cm a significantly dilated aorta to allow for a planned
in class IV. 9 Because of increased risk of aortic dissection, delivery.2,9
pregnancy is avoided when the aortic root diameter is >4.5 4. There are no randomized trials of delivery methods in
cm.1-3,9 Type B aortic dissection is responsible for 20% to women with aortopathy. When the aorta is not signif-
40% of pregnancy-related dissections in Marfan syn- icantly dilated, vaginal delivery using methods to
drome, often occurring without aortic dilation1,6,8,10 and lessen hemodynamic stress, including regional anes-
may occur after previous aortic root replacement in Marfan thesia and an expedited second stage and assisted
syndrome and Loeys-Dietz syndrome. 11,12 Aortic dissection delivery, is often performed. 2,8,9 Coexistent lumbosa-
frequently occurs postpartum, with heightened risk up to cral dural ectasia, spine disease, or both in women with
12 weeks after delivery. 1 Patients at risk and their care aortopathic conditions may complicate epidural
teams should remain alert to signs and symptoms sug- anesthesia.13,14
gesting acute dissection. 5. Cesarean delivery is often performed in women with
Marfan syndrome and aortic dilation of >4.0 cm.2,8
Among 27 women with Marfan syndrome and aortic
1. Very limited information exists about pregnancy- dilation, 21 of 27 women had a vaginal delivery. The
related aortic risks in patients with chronic aortic cesarean delivery rate was 23.8% and 16.7% in women
dissection. Because of concerns for aneurysmal with diameter <4.0 cm and 4.0 cm to 4.5 cm,
enlargement, recurrent dissection and aortic rupture, respectively.8
pregnancy is considered to be high risk in women with
chronic aortic dissection. To allow optimal timing of
8.3. Surgery Before Pregnancy in Women With Aortic Disease
delivery, elective cesarean delivery is usually per-
formed in women with chronic aortic dissection.
Recommendations for Surgery Before Pregnancy in Women With Aortic Disease
1. In patients with Marfan syndrome and an aortic root diameter of >4.5 cm, aortic surgery before pregnancy
1 C-LD is recommended.1-4
If the aortic root diameter is 4.0 cm to 4.5 cm, aortic surgery before pregnancy may be considered,
2b C-LD especially if there are risk factors for aortic dissection (ie, rapid aortic growth of ‡0.3 cm/y or a family
history of aortic dissection). 1,2,5-8
2. In patients with Loeys-Dietz syndrome attributable to pathogenic variants in TGFB2 or TGFB3 and an
2a C-EO aortic diameter of ‡4.5 cm, surgery before pregnancy is reasonable.
If the Loeys-Dietz syndrome is attributable to pathogenic variants in TGFBR1, TGFBR2, or SMAD3, and the
2b C-EO aortic diameter is ‡4.0 cm, surgery before pregnancy may be considered.
(Continued)
3. In patients with nsHTAD and an aortic diameter of ‡4.5 cm, surgery before pregnancy is recommended.
1 C-EO
If the aortic diameter is 4.0 cm to 4.4 cm, surgery before pregnancy may be considered, depending on the
2b C-EO molecular diagnosis, family history, and aortic growth rate.
4. In patients with Turner syndrome and ASI of ‡2.5 cm/m2 , surgery before pregnancy is recommended. 9-11
1 C-LD
5. In patients with a BAV (in the absence of Turner syndrome or an HTAD) and an aortic diameter of ‡5.0 cm,
1 C-EO surgery before pregnancy is recommended.
6. In patients with sporadic aortic root aneurysms, ascending aortic aneurysms, or both and a diameter
1 C-EO of ‡5.0 cm, surgery before pregnancy is recommended.
Synopsis Loeys-Dietz syndrome, because most women who were

The decision to proceed with operative intervention for pregnant were unaware of their diagnosis before
an aortic root, ascending aortic aneurysm, or both in a pregnancy. The size threshold for elective surgery to
woman contemplating pregnancy is complex and depends replace the dilated aortic root and ascending aorta in
on many factors. Considerations that inform this decision Loeys-Dietz syndrome depends on multiple factors and
include the specific disorder, genetic variant, rate of is informed by the specific pathogenic variant and the
aortic growth, family history, and phenotype and include family history, rate of aortic growth, extra-aortic
shared decision-making (Table 34). Specialists involved in phenotypic features, and involves shared decision-
this decision may include aortopathic specialists, cardi- making. Patients with TGFB2- and TGFB3-related
ologists, medical geneticists, maternal fetal medicine Loeys-Dietz syndrome may have a lower aortic
specialists, and aortic surgeons at experienced centers. dissection risk than those with variants in TGFBR1,
The risks of aortic surgery should be considered and TGFBR2, or SMAD3.16,17 Women with aortic root di-
although prophylactic aneurysm surgery will prevent ameters of >4.0 cm are likely at increased risk for
proximal aortic dissection, a risk remains of pregnancy- pregnancy-related aortic dissection based on data from
related dissection distal to the aortic graft in HTAD, and women with Marfan syndrome and the more severe
this risk may be higher in women with Loeys-Dietz syn- aortopathy in Loeys-Dietz syndrome attributable to
drome attributable to pathogenic variants in TGFBR1 and TGFBR1, TGFBR2, and SMAD3 variants.5,18-20 There
12,13
TGFBR2. were no pregnancy-related aortic dissection reported
in a series of women with SMAD3 variants, but only 2
women had aortic diameters known before pregnancy
1. Women with Marfan syndrome and aortic root dila- (and both were normal). 20
tion >40 mm and especially >4.5 cm are at increased 3. Because phenotypic features are absent in patients
risk of type A aortic dissection during pregnancy and with nsHTAD because of pathogenic variants in mul-
postpartum. 1,2,6,7,12 Pregnancy in small series of tiple genes (eg, ACTA2, MYH11, MYLK, PRKG1, and
women with Marfan syndrome and aortic diameters others), the first manifestation of disease may be acute
between 4.0 cm and 4.5 cm was reported to be aortic dissection, including that related to pregnancy.21
1-4
relatively safe in carefully monitored women, In a series of patients with ACTA2 pathogenic variants,
although acute type A aortic dissection may occur.5 20% of aortic dissection were related to pregnancy.21
The presence of additional risk factors for aortic Aortic dissection at small aortic diameters has been
dissection, including family history of aortic dissec- reported related to pregnancy in patients with ACTA2-
tion and rapid aortic growth ($0.3 cm/y), and patient and MYLK-related HTAD.21,22 Ruptured type B dissec-
preference may inform the shared decision for aortic tion has been reported. 23 Individualized assessment of
surgery before pregnancy when the aortic diameter pregnancy risks based on the specific genetic condition
is <4.5 cm. 8,14,15 and other individual factors may inform pregnancy
2. Information is lacking about aortic diameters management, recognizing that limited information is
and aortic dissection risk related to pregnancy in available to guide decision-making. 21,22,24
in 186 deliveries. 26 In a series of 49 patients with BAV

TABLE 34 Prophylactic Aortic Surgery Before Pregnancy
with moderate aortic dilation (median aortic diameter
in Women With Aortopathic Conditions
42 mm) reporting pregnancy outcomes, there were no
Surgical Threshold Before
Pregnancy* by Aortic Diameter
cases of aortic dissection. 3 When type A aortic dissec-
Condition (cm) or Aortic Size Index (cm/m 2 ) tion did complicate pregnancy in isolated BAV, signif-
Marfan syndrome >4.5 cm icant aortic dilation was noted.5,25 There is no
Marfan syndrome with risk factors 4.0–4.5 cm evidence-based information regarding pregnancy out-
(rapid aortic growth of $0.3 cm/y;
family history of aortic dissection)
comes in women with BAV and aortic diameters >4.5
cm to inform aortic risk. In these cases, pregnancy
Loeys-Dietz syndrome (attributable to $4.0 cm
pathogenic variants in TGFBR1, management and shared decisions about aortic surgery
TGFBR2, or SMAD3)
may be informed by other risk factors for dissection,
Loeys-Dietz syndrome (attributable $4.5 cm including rapid aortic growth, body size, and family
to pathogenic variants in TGFB2
or TGFB3) history. Aortic dissection risk increases in patients with
Nonsyndromic heritable thoracic $4.5 cm† BAV when the aortic diameter exceeds 5 cm.27 Because
aortic disease of risk of aortic dissection, pregnancy in patients
Turner syndrome $2.5 cm/m2 with a BAV and an aortic diameter of >5.0 cm is clas-
Bicuspid aortic valve $5.0 cm‡ sified to be modified World Health Organization class
IV, carrying high risk of maternal morbidity and
*Shared decision-making is required to determine the surgical threshold before elective
aortic root, ascending aortic surgery, or both and is informed by the condition, specific mortality. 28
pathogenic variant, age, body size, aortic growth rate, phenotype, and family history of 6. Aortic root and or ascending aortic dilation >4.0 cm in
aortic dissection, and surgery at smaller aortic diameters may be considered depending
on individual circumstances. a woman of child-bearing age is uncommon, and its
†Aortic dissection related to pregnancy has occurred at small aortic diameters in women presence should trigger an evaluation for underlying
with ACTA2 and MYLK pathogenic variants. Prophylactic aortic surgery before preg-
nancy at smaller aortic diameters may be reasonable in these conditions and other genetic aortopathy.29 Even when there is clear evi-
nonsyndromic heritable thoracic aortic disease and may be informed by the molecular dence of an autosomal dominant transmission of TAA
diagnosis, family history, and aortic growth rate.
‡Prophylactic aortic surgery may be considered at smaller aortic diameters depending in a family, currently available molecular genetic
on body size, aortic growth rate, and family history. technology only identifies a pathogenic variant in a
Colors correspond to Class of Recommendations in Table 2.
known gene leading to TAA in about 20% to 25% of
families.29 In sporadic TAA disease, genetic variants
are found in even fewer cases. In young patients at low
4. Among those with Turner syndrome, an ASI >2.0 cm/
surgical risk with aortic root or ascending aortic an-
m 2 is considered dilated, and the risk of aortic dissec-
eurysms of 5.0 cm, surgical intervention is performed.
tion in Turner syndrome is greatest when the ASI
Surgery before pregnancy at smaller aortic diameters is
is $2.5 cm/m 2. 9,10 When aortic dissection occurs in
sometimes performed and is informed by aortic growth
Turner syndrome, almost 90% of cases have an iden-
rate, hypertension, surgical expertise, patient wishes,
tifiable risk factor, such as underlying aortic dilation,
and other factors involving a shared decision depend-
aortic coarctation, BAV, or hypertension. 11
ing on individual circumstances.
5. Despite the relative frequency of BAV in the popula-
tion, aortic dissection related to pregnancy in patients
with a BAV (and without Turner syndrome or HTAD) is 8.4. Pregnancy in Patients With Aortopathy: Aortic Dissection
rarely reported.5,25 In 88 women with BAV and without and Aortic Surgery in Pregnancy
aortic dilation, there were no cases of aortic dissection
Recommendations for Pregnancy in Patients With Aortopathy: Aortic Dissection and Aortic Surgery in Pregnancy
1. In patients experiencing an acute type A aortic dissection during the first or second trimester of preg-
1 C-LD nancy, urgent aortic surgery with fetal monitoring is recommended. 1-3
2. In patients experiencing an acute type A aortic dissection during the third trimester of pregnancy, urgent
1 C-LD cesarean delivery immediately followed by aortic surgery is recommended. 1-4
(Continued)
3. In patients experiencing an acute type B aortic dissection during pregnancy, medical therapy is
1 C-EO recommended, unless endovascular or surgical therapy is required to manage acute complications.5
4. In patients with progressive aortic dilation during pregnancy, prophylactic aortic surgery may be
2b C-EO considered, depending on individual circumstances. 1,2,4
Synopsis with fetal monitoring and modifications to anesthesia

During pregnancy, if marked aortic dilation is present and cardiopulmonary bypass, recognizing the high risk
or rapid aortic expansion occurs, risks of maternal aortic of fetal loss.1-4 If acute type A aortic dissection occurs
dissection or rupture must be considered. If early in between 24 and 28 weeks, the care team must balance
pregnancy, high maternal risk of morbidity or death maternal and fetal risks when deciding between ce-
may warrant pregnancy termination. 1,4 Prophylactic sarean delivery followed by aortic surgery or aortic
aortic surgery during pregnancy requires complex surgery with fetal surveillance. 1,4
decision-making and should be individualized based on 2. If type A aortic dissection occurs in the third trimester,
maternal and fetal risks. 1,2,4 Cardiac surgery in the first given the increased likelihood of independent fetal
trimester has risks of fetal developmental defects, while survival, emergency cesarean delivery followed by
surgery in the third trimester carries risks to fetal cir- maternal aortic surgery is recommended. 1,2,4 In a series
1
culation and maternal hemodynamics. Semi-elective of 20 patients with type A aortic dissection during
surgery during pregnancy may have its lowest collec- pregnancy, 19 underwent surgical repair and, of those
tive risk to fetal organogenesis and maternal hemody- at >28 weeks gestation, delivery first followed by aortic
namics during the second trimester.1,3,4 If type A aortic surgery achieved good fetal outcomes.2
dissection occurs during pregnancy, urgent obstetric 3. Although uncomplicated type B aortic dissection in
and cardiac surgical consultation is necessary, because pregnancy is treated medically, 20% will go on to
management strategies depend on the viability of the develop complications that require intervention 5; in
fetus and condition of the mother. If type A aortic such cases, endovascular repair is preferred over open
dissection occurs in the first 26 weeks, emergency car- surgery, whenever feasible. 5
diac surgery is performed, recognizing risk of fetal 4. Prophylactic aortic surgery during pregnancy requires
loss.1,2,4 When duration of pregnancy associates with complex decision-making, and management is indi-
higher likelihood of independent fetal survival (espe- vidualized based on maternal and fetal risks and
cially after 28 weeks), cesarean delivery followed by benefits.1,2,4
aortic repair provides the best chances for fetal and
maternal survival.1,2,4
9. OTHER AORTIC CONDITIONS
9.1. Inflammatory Aortitis: Diagnosis and Treatment of
1. If type A aortic dissection occurs during the first 2 tri-
Takayasu Arteritis and Giant Cell Arteritis (GCA)
mesters, emergency aortic surgery is performed first
Recommendations for Inflammatory Aortitis: Diagnosis and Treatment of Takayasu Arteritis and GCA
Diagnosis
1. In patients with large vessel vasculitis (LVV), prompt evaluation of the entire aorta and branch vessels
1 C-LD with MRI or CT, with or without 18F-FDG positron emission tomography (FDG-PET), is recommended. 1-6
(Continued)
Treatment
2. In patients with active GCA or Takayasu arteritis, initial medical therapy should include high-dose glu-
1 B-NR cocorticoids. 7-12
3. In patients with GCA who have evidence of active aortitis, tocilizumab is recommended as adjunctive
1 B-R therapy to glucocorticoids, with methotrexate as an alternative.7,13,14
4. In all patients with Takayasu arteritis, nonbiological disease-modifying anti-rheumatic drugs (DMARD)
1 C-LD should be given in combination with glucocorticoids. 7,15,16
5. In patients with active GCA or Takayasu arteritis, treatment efficacy should be periodically assessed by
1 C-LD monitoring inflammatory serum markers (C-reactive protein and erythrocyte sedimentation rate),
imaging with CT, MRI, or FDG-PET, and clinical symptoms. 1,7,15,17-20
6. In patients with GCA or Takayasu arteritis who are in remission, elective endovascular or open surgical
2a C-LD intervention is reasonable to treat aortic and branch vessel complications. 7,21
7. In patients with GCA or Takayasu arteritis and aortic involvement who are in remission, annual
2a C-EO surveillance imaging with CT, MRI, or FDG-PET is reasonable. 1,7,17-19
Synopsis complement clinical findings.1 Imaging the aorta

LVV comprises Takayasu arteritis and GCA, which are should be performed as soon as possible so that initi-
the most common causes of aortitis.22,23 Other known ation of treatment is not delayed, given the risk of
causes of aortitis include immunoglobulin G4–related complications from untreated LVV. Sensitivity of
disease, antineutrophil cytoplasmic antibody-related diagnostic imaging in the initial diagnosis of LVV de-
vasculitis, sarcoidosis, Behçet’s disease, relapsing poly- creases with duration of glucocorticoid treatment. 2
chondritis, and granulomatosis with polyangiitis; many FDG-PET has a reported specificity for GCA-related
cases of aortitis remain idiopathic. Whereas Takayasu aortitis as high as 100% and CTA about 85%.5 In CT,
arteritis and GCA tend to affect the thoracic aorta, wall thickening from inflammation may be mistaken
immunoglobulin G4–related disease most commonly af- for atherosclerosis; however, given CT’s usefulness in
fects the abdominal aorta. Diagnostic criteria are sum- assessing occlusive lesions, intimal injury, ulcerative
marized in Table 35. Prompt diagnosis and initiation of plaques, and aneurysmal disease, it is often combined
treatment is of utmost importance, because potential with FDG-PET in LVV.1 Evidence is limited for the role
complications include aortic aneurysms, aortic dissection, of MRI in GCA, but MRI is widely used in Takayasu
IMH, PAU, and rupture, as well as progressive athero- arteritis given the patients’ younger age at diagnosis
sclerosis and thrombotic complications.24 18F-FDG-PET and need for lifelong surveillance imaging. 6 If proximal
with CT is useful for both the diagnosis of suspected LVV aortic involvement is confirmed by CT or MRI, then
and to evaluate anti-inflammatory treatment response, echocardiography may be helpful to assess aortic valve
especially before planned revascularization.4,5 Initial function.
treatment options for Takayasu arteritis and GCA include
2. Active vasculitis is diagnosed by clinical symptoms of
high-dose glucocorticoid therapy (prednisone at 40–60
GCA or Takayasu arteritis with evidence of inflamma-
mg/d, or equivalent) or, for select cases, intravenous pulse
tion by serum biomarkers, imaging, or both. High-dose
steroids along with adjunctive therapy, including (but
glucocorticoid therapy (prednisone at 40–60 mg/d or
not limited to) tocilizumab and methotrexate (Figures 25
equivalent) is standard induction therapy for GCA and
and 26). Revascularization may be warranted in select
Takayasu arteritis and leads to remission and control of
cases of stable disease, as well as in AAS.
active disease in most patients 7-12 (Figures 25 and 26).
Evidence supporting the efficacy of induction therapy
1. In suspected GCA or Takayasu arteritis, early imaging with high-dose intravenous methylprednisolone
can confirm the diagnosis when the results in GCA comes only from small clinical trials, and thus
TABLE 35 Diagnostic Criteria for Inflammatory Aortitis
Names Criteria Used in Diagnosis When Is Diagnosis Established?

Takayasu arteritis Age of onset <40 y $3 criteria are present (sensitivity 90.5%; specificity 97.8%)
Intermittent claudication
Diminished brachial artery pulse
Subclavian artery or aortic bruit
Systolic BP variation of >10 mm Hg between arms
Aortographic evidence of aorta or aortic branch stenosis
Giant cell arteritis Age >50 y $3 criteria are present (sensitivity >90%; specificity >90%)
Recent-onset localized headache
Temporal artery tenderness or pulse attenuation
Elevated erythrocyte sedimentation rate >50 mm/h
Arterial biopsy shows necrotizing vasculitis
Reprinted from Hiratzka et al. 2019.27
the 2018 recommendations from the European Alli- is to initiate DMARDs in combination with glucocorti-
ance of Associations for Rheumatology (EULAR; coids in all patients with Takayasu arteritis, given high
formerly the European League Against Rheumatism) relapse rates of up to 70%. 7 Nonbiological DMARDs (eg,
limits its use to patients with severe GCA at risk for methotrexate, hydroxychloroquine, azathioprine, sul-
blindness in the acute setting, and administration famethoxazole, and leflunomide) are considered first
should not delay oral glucocorticoid treatment.7-9 line according to the EULAR 2018 updated guidelines
Once the acute phase is controlled, glucocorticoid on Takayasu arteritis treatment, with biological
taper should be initiated to reach a target prednisone DMARDs (eg, tocilizumab or tumor necrosis factor-
dose of 15 to 20 mg/d within 2 to 3 months, and #5 inhibitors) as second-line agents in select patients
mg/d for GCA and #10 mg/d for Takayasu arteritis who relapse on initial combination therapy 7,15,16
7
after 1 year. Older guidelines have supported the use (Figure 26). Optimal treatment duration in Takayasu
of antiplatelet or anticoagulants in LVV. Evidence arteritis is less well understood, because defining
from a meta-analysis does not support use of pro- remission in Takayasu arteritis is challenging. Out-
phylactic antithrombotic therapy in all patients with comes measures may include any of these: remission
GCA25; instead, an individualized approach to antith- based on clinical criteria, normalization of inflamma-
rombotic therapy is recommended in the acute and tory biomarkers, stabilization on serial CT or MRI,
chronic phases of GCA and Takayasu arteritis, based improvement on PET-CT imaging, quality of life, and
on imaging and clinical findings of aortic and branch presence of clinical disease relapse. 15 A clear need re-
26
vessel complications. mains for both adequately powered randomized clin-
3. In an RCT of 251 patients with GCA, a 26-week pred- ical trials of Takayasu arteritis therapies and a
nisone taper combined with tocilizumab, an consensus definition of treatment success.
interleukin-6 receptor inhibitor, was superior to either 5. The EULAR 2018 updated guidelines placed the great-
a 26-week or 52-week prednisone taper plus placebo in est emphasis on both the improvement of clinical
reducing the primary outcome of glucocorticoid-free symptoms and the stability of inflammatory bio-
disease remission at 1 year.10 Tocilizumab gained markers in defining the remission phase of LVV.
approval for use in 2017 as adjunctive therapy for select Consequently, data are limited regarding the role of
patients with GCA, with methotrexate remaining an surveillance imaging in those with no signs or symp-
alternative option.7,13,14 The EULAR 2018 updated toms of active disease. Currently, tomographic imaging
guidelines recommended limiting the use of adjunctive is complementary to clinical symptoms and laboratory
therapy to those with refractory or relapsing disease, surveillance, and its use should be individualized,
those at risk of adverse effects of glucocorticoid treat- focused mostly on the evaluation of new symptoms or
ment, or those at risk of cardiovascular complications signs of aortic, major branch artery stenoses or aneu-
(aortitis and major branch vessel involvement) from rysms, or both.1,7,15,17-19 One prospective cohort study
7
GCA (Figure 25). using FDG-PET in disease surveillance of GCA showed
4. High-quality randomized clinical trial evidence sup- reduced inflammatory activity at 3 months after treat-
porting the use of adjunctive therapy in Takayasu ment initiation but no further change at 6 months, with
arteritis is limited. However, consensus expert opinion most patients in clinical remission still showing
F I G U R E 2 5 The 2018 European Alliance of Associations for Rheumatology (EULAR; formerly European League Against Rheumatism) Recommended Algorithms
for the Pharmacological Treatment of Giant Cell Arteritis
GC indicates glucocorticoids; GCA, giant cell arteritis; and TNF, tumor necrosis factor. Modified from Hellmich et al.7 Copyright 2020, with permission from BMJ
Publishing Group Limited.
F I G U R E 2 6 The 2018 European Alliance of Associations for Rheumatology (EULAR; formerly European League Against Rheumatism) Recommended Algorithms
for the Pharmacological Treatment of Takayasu Arteritis
csDMARD indicates conventional synthetic disease modifying antirheumatic drug; GC, glucocorticoids; and TNF, tumor necrosis factor. Modified from Hellmich et al.7
Copyright 2020, with permission from BMJ Publishing Group Limited.
positive PET findings.20 What remains unknown are evidence of active aortic inflammation resulting in
the potential anatomic consequences of having a pos- branch vessel stenosis, aortic aneurysm, or dissection.
itive FDG-PET scan despite clinical remission. Remission of LVV is characterized by lack of new
6. In patients with LVV who are in remission and have clinical symptoms, a normalization of inflammatory
aortic or branch artery complications that do not war- biomarkers, and no evidence of progressive aortic and
rant urgent intervention, the role of elective endovas- branch artery dilation or narrowing by surveillance
cular or open surgical repair approach should be imaging. However, signals of vessel inflammation may
determined by a multidisciplinary team including, but persist even in the absence of clinical disease.1,6,19 For
not limited to, vascular surgery, vascular medicine, those in remission, annual surveillance imaging with
cardiology, and radiology specialists. The risk of such CT or MRI is useful to detect disease progression in the
elective intervention is lowest when patients are in the aortic and branch arteries, even in the absence of
remission phase of the LVV 7; therefore, before inter- inflammation. More frequent surveillance imaging may
vention, imaging with 18F-FDG-PET CT is often helpful be necessary when evidence of active disease pro-
to assess treatment response and quantify the degree gression is apparent on annual imaging or if new
of ongoing active inflammation.1,4,5,18 symptoms suggestive of arterial stenosis arise.
7. The EULAR consensus definitions for relapse and
remission have been incorporated into the 2018 upda-
9.2. Infectious Aortitis
ted recommendations for management of LVV.7 A
9.2.1. Diagnosis and Management of Infection of the
major relapse of GCA and Takayasu arteritis includes
Native Aorta
recurrence of clinical features of ischemia (ie, visual
loss, jaw claudication, limb claudication, stroke) or
Recommendations for Diagnosis and Management of Infection of the Native Aorta
1. In patients with infectious aortitis and associated aneurysms or dissection of the thoracic or abdominal
1 C-EO aorta, open surgical repair is recommended.
In select patients, treatment with endovascular repair may be considered. 1-3

2b C-LD
2. In patients with infectious aortitis complicated by rupture, either open or endovascular repair is
2a C-EO reasonable, based on the patient’s status at presentation and institutional expertise.
3. In patients with infectious aortitis, intravenous antimicrobial therapy of at least 6 weeks’ duration may be
2b C-EO considered, with lifelong suppressive therapy in select cases not amenable to interventional repair or who
have recurrent infection.
Synopsis Medical therapy is challenging because the causative

The term “infectious aortitis” describes an infection of organism is not always identified, but a prolonged
the aorta and has supplanted the older term “mycotic course of antibiotics is often warranted. 4 The mortality
aneurysm,” which was used broadly but actually implies a rate of infectious aortitis is high, because complications
fungal cause. Aortic infections arise from either contig- include sepsis, aneurysm formation (saccular or pseu-
uous spread from adjacent structures or septic emboli and doaneurysm), erosion and subsequent fistula, dissection,
hematogenous spread of microorganisms to the aortic wall or rupture. CT and MRI can size the aneurysm, detect
via a vulnerable plaque or preexisting aneurysm.4 Staph- complications, and aid in interventional planning. TEE
ylococcus aureus, Pneumococcus, Escherichia coli, and Sal- is especially useful for imaging involvement of the
monella are the pathogens identified in most reports.1-6 aortic root and associated complications.5 Open surgical
Syphilitic aortitis, which typically appears 10 to 25 years repair is the standard treatment for infectious aortitis;
after systemic Treponema pallidum infection, is now rare. however, in select patients with rupture, fistula, hemo-
Fungal aortitis (from Candida or Aspergillus) and tuber- dynamic instability, or both, a hybrid or bridging
culous aortitis are uncommon and typically arise in approach with endovascular therapy (Table 36) may be
immunocompromised hosts. used.2-8
Management of Aortic Mycotic Aneurysm: Comparison of Resection and Extra-Anatomic Reconstruction, In Situ
TABLE 36
Reconstruction, or Endovascular Device Repair
Procedure Potential Indications* Advantages Disadvantages

Extra-anatomic Infrarenal location with gross purulence, Avoids placement of foreign body in Not technically feasible for thoracic, suprarenal, or
reconstruction psoas or retroperitoneal abscess, infected area visceral location or for emergency use
vertebral osteomyelitis, inadequate Long operating time
response to antibiotic therapy, Long-term patency rates low
selected aortoenteric fistulae Stump blowout
Limb ischemia, amputation
Reinfection rate higher than for in situ reconstruction
Ischemic colitis
In situ reconstruction Thoracic, suprarenal, infrarenal, More versatile than extra-anatomic: fewer Theoretical risk of infection because of interposition
or visceral location long-term complications, higher patency of foreign material in infected site
Selected aortoenteric fistulae rates, lower recurrent infection rate,
shorter operating time
Polyester grafts† available for emergency
surgery
Endovascular device Bridge procedure‡: hemodynamic Emergency stabilization Persistent infections and device infections
repair instability, uncontrolled bleeding, Low early morbidity, mortality Less Higher long-term morbidity, mortality with
rupture or impending rupture, invasive device retention
selected patients with aortocentric No cross-clamping of aorta: spinal Requires device explanation, reconstruction
fistulae, patients who are not fit cord injury, reperfusion injury
for open surgery
*Potential indication; must be individualized for each patient.

†Polyester grafts, rifampin-soaked or silver-coated; less experience reported with cryopreserved arterial allografts or venous autografts.
‡Bridge procedure, used to stabilize patients until device explanation and arterial reconstruction.
Adapted from Wilson et al5 with permission of the American Heart Association, Inc. Copyright 2016 American Heart Association, Inc.
Recommendation-Specific Supportive Text was not associated with an increase in infection-related

complications or a need for late reoperation. 3 Use of
1. A diagnosis of infectious aortitis or mycotic aneurysm
endovascular repair in the management of infectious
and its complications warrants prolonged antimicrobial
thoracic aneurysms, abdominal aneurysms, or both
therapy regardless of intervention, with 2016 scientific
warrants ongoing study, and at present may be most
statement from the AHA suggesting a duration of 6
appropriate as a bridge procedure in cases of instability
weeks to 6 months, with consideration of lifelong
or impending rupture, or in patients who may not be fit
suppressive therapy in some cases.5 Given the high risk
for open surgical intervention 5 (Table 36).
of rupture or contained rupture in infectious aortitis,
open surgical repair is often warranted, although the 2. The prognosis is often poor for infectious aortitis,
data supporting open surgical repair are limited, with especially if rupture has occurred. 6 From a large single-
most evidence derived from single-institution case institution study over 18 years of 2,520 patients who
series and small cohort studies. 6-8
Open surgical repair underwent surgery for infectious aortic aneurysms,
includes in situ reconstruction or aortic resection with 24% of aneurysms had already ruptured at presenta-
extra-anatomic bypass (ie, axillobifemoral bypass or tion, and 61% had penetrated into periaortic tissues. 6
femorofemoral crossover bypass graft placement) 3; Open surgical treatment options include resection of
surgical debridement of all infected tissue is essential infected aorta with extra-anatomic reconstruction
to minimize the risk of persistent infection. The use of (for abdominal aneurysm), or in situ reconstruction
endovascular repair has been increasing in select pa- (for thoracic aneurysms and some aortoenteric
tients with infectious aortitis. 6-8
Limited data are fistulae).2-5,7,8 The choice of intervention is based on
available for comparison of open surgical versus multiple factors (Table 36), including the location and
endovascular repair; some small studies showed extension of the aneurysm(s), the presence of fistulae,
similar long-term survival between the 2 methods in and the patient’s clinical status. In select patients with
treatment of infectious abdominal aortitis,1-4 although aneurysm rupture and hemodynamic instability and/or
the evidence may have selection bias. In a nationwide uncontrolled bleeding, endovascular repair may be
Swedish retrospective population-based cohort study used. 6
of 132 patients, of whom 50 (38%) presented with 3. Because peripheral blood cultures and surgical spec-
rupture, using propensity score analyses, 5-year sur- imen cultures may be negative in a large proportion of
vival was similar with open repair versus EVAR, at 60% patients with infectious aortitis, 5 the choice of anti-
versus 58%, respectively.3 Moreover, the use of EVAR microbial agents may be empiric, and infectious dis-
was associated with improved short-term survival and ease experts are usually involved in directing therapy.
Treatment with antimicrobial therapy alone (ie, without possibly longer. 5,11 Because the response of uncompli-
intervention) is associated with high mortality rate and cated (without rupture or fistulae) infectious aortitis to
may not prevent aneurysm expansion or rupture 6,9,10 antimicrobial therapy may influence the choice of
and is thus reserved for patients who are not candi- interventional approach, it is also reasonable to have
dates for open or endovascular repair or for those in patients undergo surveillance imaging at intervals
whom a palliative approach is appropriate. No clinical deemed appropriate by a multidisciplinary care team.
trial data are available to define the optimal duration of
antimicrobial therapy, whether as solo therapy or as
9.2.2. Diagnosis and Management of Prosthetic Aortic
adjunctive therapy to aortic intervention, but expert
Graft Infection
opinion suggests a duration of at least 6 weeks, and
Recommendations for Diagnosis and Management of Prosthetic Aortic Graft Infection

fmkReferenced studies that support the recommendations are summarized in the Online Data Supplement.
Diagnosis
1. In patients with a prosthetic aortic graft, who have signs and symptoms or culture evidence of unex-
2a B-NR plained infection or have unexplained gastrointestinal bleeding, cross-sectional imaging is reasonable to
evaluate for an underlying aortic graft infection. 1-6
Treatment
2. In patients with an infected prosthetic aortic graft who are hemodynamically stable and have appropriate
2a B-NR anatomy, it is reasonable to perform open surgery with either in situ reconstruction or extra-anatomic
bypass. 7-13
3. In patients with an infected prosthetic aortic graft who are hemodynamically unstable, it is reasonable to
2a B-NR perform open surgery with either explant or in situ reconstruction.7
4. In patients with an infected prosthetic aortic graft, endovascular therapy is reasonable, either as bridge
2a C-LD therapy in those with hemodynamic instability or as long-term therapy in those who are unsuitable
candidates for open surgery.13-15
Late Management
5. In patients who have undergone treatment of an acute prosthetic aortic graft infection, targeted intra-
1 C-LD venous antimicrobial therapy of at least 6 weeks’ duration, with prolonged suppressive oral therapy in
select cases, plus a consultation and follow-up with an infectious disease specialist, is
recommended. 7,11,12,16,17
6. In patients with an infected prosthetic aortic graft and either an extensive perigraft abscess or an
2b C-LD infection caused by methicillin-resistant S. aureus, Pseudomonas aeruginosa, or a multidrug-resistant
microorganism, or who have undergone in situ reconstruction, lifelong suppressive oral antimicrobial
therapy may be considered after the initial course of therapy. 14,15,18,19
Synopsis Aortic graft infection is uncommon (0.3%–3%).20-22

Recommendations in this section apply to prosthetic Extension to the groin increases the risk of subsequent
aortic grafts. This includes tube grafts placed via open infection. Although some studies suggest a lower risk with
surgery as well as endovascular stent grafts. Although endovascular versus open repair, the EVAR-1 (UK Endo-
these grafts are typically made with Dacron or polytetra- vascular Aneurysm Repair 1) RCT and a large Medicare
fluoroethylene, these recommendations also apply to al- analysis found equivalent rates of graft infection. 23-25
lografts (eg, cryopreserved aorta) and autografts (eg, Common sources of infection include: contamination at
femoral vein). the time of implantation; graft enteric erosion or fistula to
adjacent bowel, esophagus, or airway; or, rarely, hema- the lowest rate of reinfection but is associated with
togenous spread from remote infection. Suspicion is long operative times, size mismatch, and lower ex-
usually raised by symptoms, laboratory test abnormal- tremity venous morbidity.7,9 Cryopreserved allografts
ities, or axial imaging findings. In the presence of an have a low rate of reinfection (similar to vein) but are
aortic graft infection, no surgical option is clearly supe- susceptible to early and late degeneration, may have
rior. Basic tenets are to remove all infected tissue, limited lengths and diameters, and have limited
including the graft and surrounding tissue, reconstruction availability for emergencies.7,10,11 Rifampin- or silver-
of distal flow either as an extra-anatomic or in situ bypass, impregnated prosthetic grafts are more readily avail-
and coverage of the contaminated field with omentum, able and faster to implant than vein or extra-anatomic
muscle flaps, or pleura. Previously, extra-anatomic repair but are more susceptible to reinfection.7,26
bypass followed 24 to 48 hours later by graft explant None of these graft options is clearly superior to the
and oversewing of the aortic stump was considered the others and, as such, in the stable patient without
gold standard for abdominal aortic infection but is usually extensive infection with resistant organisms, the use
not appropriate for the thoracic aorta. Aortic allografts, of any of these is acceptable. 27 For those with exten-
deep vein, and silver-impregnated or rifampin-soaked sive peri-graft abscess, or infection with methicillin-
prosthetic grafts placed in situ have all shown good re- resistant S. aureus, Pseudomonas, or multidrug
sults as well, often with lower complication rates. A 6- resistant organisms, extra-anatomic reconstruction
week course of intravenous antibiotics is typically used, (when feasible) or in situ reconstruction with femoral
sometimes followed by long-term oral suppressive vein or allograft may offer improved freedom from
therapy. reinfection.7,13,26
3. Hemodynamically unstable patients require emer-
gency proximal control with a clamp or balloon, and
1. Early graft infection (#3 mo) is often associated with rapid in-line reconstruction, which is best performed
fever and back pain, whereas late graft infections (>3 with either an allograft (if immediately available) or a
mo) may have an insidious onset with symptoms of silver- or rifampin-impregnated prosthetic graft.7
fatigue and malaise, or may have fever, an elevated 4. Endovascular intervention allows relatively rapid
white blood cell count, erythrocyte sedimentation rate, control of hemorrhage and may improve survival in
C-reactive protein, or advanced signs of sepsis with patients with an aorto-enteric fistula, when used as a
hemodynamic instability or frank hemorrhage from bridge to definitive therapy. 13-15 For patients who are
rupture or fistulae to adjacent bowel, esophagus, or not candidates for surgical graft excision, endovascular
airway. Because these signs and symptoms are therapy may be considered for definitive therapy, in
nonspecific for site of infection, the initial workup which case lifelong antibiotic suppression should be
should include basic blood work, blood cultures, and considered.
axial imaging, preferably with CTA. In those patients 5. Consultation with an infectious disease specialist is
with bleeding, endoscopy may be used to rule out recommended for all patients with aortic graft infec-
other causes and potentially temporize bleeding. tion. A 6-week course of intravenous antimicrobial
Findings of graft infection on CT include peri-graft air, therapy has been recommended in multiple reports
abscess, inflammatory changes, pseudoaneurysms, or from high-volume centers and in scientific
frank hemorrhage. CTA has a sensitivity of 94% and statements.7,11,16,17,26,28 For Pseudomonas or multidrug
specificity of 85% to 100% with advanced graft infec- resistant organisms, multiple antimicrobial agents may
tion, but the sensitivity is only 64% for those with low- be needed. A subsequent course of oral antimicrobial
grade infection.1,2 The sensitivity and specificity for therapy for 3 to 6 months may be considered depend-
low-grade infection may be increased from 77% to 93% ing on the specific organism, the extent of infection,
and 70% to 89%, respectively, with the use of PET- and the type of repair.
CT.3-5 MRI, tagged white blood cell scans, or both may 6. Lifelong suppressive oral antimicrobial therapy has
also be useful, depending on local expertise and been suggested for selected patients, such as those
availability. 6 with extensive infection, aggressive organisms, in situ
2. Extra-anatomic bypass with subsequent graft explant, prosthetic replacement, or endovascular coverage
aortic stump oversewing, and omental coverage has a without resection.14,15,18,19 Axial imaging is typically
reasonably low rate of reinfection but a relatively high continued long term to identify evidence of reinfec-
rate of amputation and occlusion and is susceptible to tion, such as inflammatory changes, fluid or air col-
stump blow-out.7,8 In situ venous reconstruction has lections, or pseudoaneurysm formation.
9.3. Atherosclerotic Disease
Recommendations for Atherosclerotic Disease
1. In patients with aortic atherosclerotic disease and concomitant coronary artery disease, PAD or both, it is
1 C-LD recommended to prescribe antiplatelet therapy, anticoagulant therapy or both, guided by the clinical
setting.1-3
2. In patients with aortic atherosclerotic disease and risk factors for confirmed coronary artery disease, it is
2a C-LD reasonable to prescribe a moderate- or high-intensity statin. 4-6
3. In patients with aortic atheromas of a thickness ‡4 mm, statin therapy may be reasonable.1,7-9
2b C-LD
Synopsis with aortic atheromatous plaques (atheromas) of $4

Atherosclerosis is a chronic immunoinflammatory, mm versus controls was 9.1 (95% CI, 3.3–25.2;
fibroproliferative disease of the aorta and its branches P<0.001).7 Moreover, in a clinical trial of 519 patients
that is propagated by lipids.10 This disease process has with severe thoracic aortic plaques, multivariate anal-
multiple risk factors and begins early in life so that the ysis showed that statin therapy was protective against
aorta may develop extensive disease over many de- strokes (P¼0.0001).8 (The data from these 2 studies
11
cades. The diagnosis of aortic atherosclerosis may occur relate specifically to atheroma thickness of $4 mm,
incidentally, during the evaluation of symptomatic which does not align precisely with the most
vascular events, or both. The size and location of aortic commonly used grading systems for severity of aortic
plaques have been associated with embolic complica- atherosclerosis, which define severe atheromas by a
tions. 4,7,8,12-16 The presence of aortic atheromas has been thickness of >5 mm.) Although antiplatelet therapy is
significantly associated with all-cause death.9 The man- commonly used in patients with aortic atheromas,
agement of aortic atherosclerosis includes, in general, there is no evidence to support the use of prophylactic
control of risk factors, lifestyle modification, and appro- anticoagulation in this population.
priate pharmacological therapies. Although lifestyle
changes may be the most important treatment strategy,
compliance may be challenging.17,18 9.3.1. Aortic Thrombus
Aortic mural thrombus is typically associated with un-
Recommendation-Specific Supporting Text
derlying aortic pathology, such as aneurysm, aortitis,
1. Patients with aortic atherosclerosis often have atherosclerosis, dissection, and aortic graft material. 1-3
concomitant cardiovascular diseases such as coronary Because such thrombi arise in the setting of underlying
artery disease, atrial fibrillation, and PAD. These aortic pathology, the thrombi can be considered “sec-
concomitant conditions frequently determine the se- ondary,” and they most often appear in the descending
lection of guideline-based antiplatelet agents, antico- thoracic and abdominal aorta.1-3 In contrast, “primary”
1-3
agulant agents, or both. thrombus occurs in a normal or minimally atherosclerotic
2. The indications for statin therapy in patients with a aorta and, rather than being mural, are often peduncu-
history of coronary artery disease, myocardial infarc- lated and protrude into the aortic lumen. Most often,
tion, and stroke are well established.5,6 The data for primary aortic thrombi are idiopathic, but some have been
statin therapy specific to aortic atherosclerosis alone associated with hypercoagulable states (eg, malignancy,
are very limited. Therefore, this recommendation has heparin-induced thrombocytopenia, and the anti-
been made for those patients at risk for or with phospholipid syndrome). 2-6
confirmed coronary artery disease because the avail- Aortic thrombus is most often asymptomatic but may
able data best support statin therapy in this cohort. present with limb ischemia, visceral ischemia, or stroke 2-7
3. Atherosclerotic disease of the aortic arch is a potential from embolization. The diagnosis is often typically
source of emboli to the brain. 1,2,9 A prospective study confirmed by either CTA or TEE.8,9 Asymptomatic pa-
(N¼500) showed that the OR for stroke among patients tients with secondary mural thrombus are usually
managed conservatively, but patients with primary aortic early follow-up. Open aortic reconstruction has improved
thrombus or those presenting with embolic events are long-term patency compared with less invasive options 3
often managed with anticoagulation, endovascular inter- but at a cost of a higher risk of perioperative
vention, or open surgical therapy; such treatments are complications.
informed by the patient’s history and the location, size,
and mobility of the thrombus. 2-7,10,11 Long-term anti- 9.3.3. Porcelain Aorta
coagulation is most often considered in patients with “Porcelain” aorta refers to the extensive, eggshell-like,
thrombus in the ascending aorta and aortic arch, because near-circumferential or circumferential calcification of
of the increased risk of stroke from potential embolization the intima or media of the aortic wall in the ascending
should aortic thrombus recur. 5-7,10 aorta or aortic arch. It is most often associated with late-
stage atherosclerosis, although it can also be a late
9.3.2. Aortic Occlusion consequence of aortitis. It generally occurs in older pa-
Aortic occlusion, which occurs most often secondary to tients with atherosclerotic disease elsewhere and carries
extensive atherosclerotic disease, can present along a an increased risk for cardiovascular events and mortality. 1
spectrum of acute and chronic clinical courses. CTA is Porcelain aorta is best seen on a noncontrast CT scan,
most useful in identifying the occlusion, determining its although very thin calcification may only be detected
cause, and defining the extent of associated aortic and intraoperatively with epi-aortic ultrasound or manual
branch arterial disease. Aortic occlusion typically occurs palpation.
below the renal arteries but rarely can arise above this Impenetrable ascending aortic calcification makes it
level, leading to renal and possibly visceral malperfusion. difficult, if not impossible, to perform central aortic can-
Treatment in acute presentations is typically surgical, nulation for cardiopulmonary bypass, the anastomosis of
including open embolectomy in the setting of embolus or proximal coronary bypass grafts to the aorta, aortotomy
aorto-iliac and femoral reconstruction for atherosclerotic during aortic valve replacement, and graft-aorta anasto-
occlusion.1 Chronic aortic occlusion can occasionally be moses during aortic replacement. Additionally, perform-
asymptomatic because collateral circulation has devel- ing aortic cross-clamping for cardiopulmonary bypass can
oped, in which case intervention may not be required. crack the calcified wall, increasing the risk of stroke from
More commonly, patients with chronic aortic occlusion embolization, or immediate exsanguination. Surgical
present with lower extremity claudication that may be management strategies have included use of alternative
accompanied by buttock claudication, central core muscle sites for cannulation and proximal bypass grafts with off-
weakness, and impotence in males caused by pelvic pump or beating heart techniques,2-4 balloon occlusion of
malperfusion. These patients often have cardiopulmo- the aorta,5 and the use of circulatory arrest with
nary comorbidities and multifocal atherosclerotic disease, ascending aortic replacement.6
and these issues should be addressed preoperatively to
mitigate potential complications. 9.4. Coarctation of the Aorta (CoA) and Congenital
Revascularization options include endovascular, 2 open Abnormalities of the Arch
aortic (eg, aortobifemoral bypass),3 or extra-anatomic (eg, CoA is a narrowing of the aorta occurring most often
axillofemoral bypass), and hybrid options (eg, iliofemoral just distal to the left subclavian artery, typically with
endarterectomy and patch plus iliac stenting). The an aneurysmal aortic segment immediately beyond the
preferred revascularization strategy is informed by the stenosis, but variants are frequent. 1 Significant CoA
arterial anatomy, the severity of disease and symptoms, presents with upper extremity hypertension and lower
the patient’s substrate, and the expected procedural extremity hypotension (Table 37). MRI and CT are both
durability. No RCTs have shown an advantage for any useful to evaluate the extent of aortic narrowing and
given revascularization procedure, and all perform well in dilation, as well as the presence of collaterals,2 whereas
TABLE 37 Criteria for Significant CoA 11,28
The presence of significant CoA is based on evidence of upper extremity hypertension (at rest, on ambulatory BP monitoring, or with pathologic blood pressure response
to exercise) or left ventricular hypertrophy and evidence for 1 of these gradient measurements:
1. A noninvasive blood pressure difference of >20 mm Hg between the upper and lower extremities
2. A peak-to-peak gradient of >20 mm Hg across the coarct by catheterization; or a peak-to-peak gradient of >10 mm Hg across the coarct by catheterization
in the setting of decreased left ventricular systolic function or significant collateral flow
3. A mean gradient of >20 mm Hg across the coarct by Doppler echocardiography; or a mean gradient of >10 mm Hg across the coarct by Doppler
echocardiography in the setting of decreased left ventricular systolic function or significant collateral flow
CoA indicates coarctation of the aorta.

TTE is useful for evaluating the gradient across the the associated cardiovascular risks and the potential
CoA, as well as identifying a coexisting BAV (present in requirement for repeat intervention.6,14
3
50%) and other potential congenital defects. Untreated An aberrant subclavian artery (ASCA) is commonly an
CoA may be complicated by aortic dissection, heart incidental finding but may present with compressive
failure, ruptured cerebral aneurysm, distal hypo- symptoms (including dysphagia and dyspnea) because it
perfusion, or the consequences of significant hyper- courses posterior to the esophagus and trachea and may
tension. Late complications following surgical or associate with aneurysm disease. 15-18 A normal left aortic
endovascular CoA repair may include undersized grafts, arch with a right ASCA occurs in w1% of the population,
recurrent stenosis, aneurysm or pseudoaneurysm for- whereas a right aortic arch with a left ASCA is much rarer
mation, and rupture, which are typically treated with and may form a vascular ring.17,18 Dilation of the origin of
endovascular procedures unless anatomic features either a right or left ASCA occurs in 20% to 60% of cases
dictate open or hybrid surgery.4-11 Hypertension is and is known as a Kommerell diverticulum. 15,18 Such
common after CoA repair, especially during exercise, Kommerell diverticula may lead to aortic dissection,
and when the repair is performed in adults.12,13 rupture, or embolization. 18-20 Indications for treatment of
Ambulatory BP monitoring and exercise testing are ASCA relate to symptoms and aneurysm size.
useful in diagnosis and management.12,13 Patients with
CoA undergo lifelong follow-up and imaging because of 9.4.1. Coarctation of the Aorta
Recommendations for CoA

1. In patients with CoA, including those who have undergone surgical or endovascular intervention, an MRI
1 B-NR or CT is recommended for initial, surveillance, and follow-up aortic imaging. 1-4
2. In patients with CoA, BPs should be measured in both arms and one of the lower extremities.
1 C-EO
3. In patients with significant native or recurrent CoA (Table 37) and hypertension, endovascular stenting or
1 B-NR open surgical repair of the coarctation is recommended. 2,3,5-12
4. In patients with CoA, guideline-directed medical therapy is recommended for the treatment of
1 C-EO hypertension. 13
5. In adult patients with CoA, screening for intracranial aneurysms by MRI or CT may be reasonable. 14-18
2b B-NR
Synopsis and hybrid procedures, depending on patient-specific and

CoA may have many anatomic variants and occurs most anatomic features.2,3,5,8-12 In patients with previous pro-
commonly at the level of the ductus arteriosus and distal to cedures, late complications may include recurrent stenosis,
the left subclavian artery. Echocardiogram is indicated in aneurysm or pseudoaneurysm formation, rupture, and
the evaluation of patients with CoA because a BAV coexists persistent hypertension. 2,3,6,8,12,21 Hypertension is com-
in at least 50% of cases, and CoA may associate with common after CoA repair, especially during exercise, and
plex congenital heart disease.4 Upper extremity hyperten- ambulatory monitoring and exercise testing may be useful
sion and lower extremity hypoperfusion are the hallmarks in diagnosis and management.3,6,7,22-24 Lifelong clinical
of CoA. Intracranial aneurysms may occur in adults with and imaging follow-up is important to evaluate for hyper-
CoA. 14-16 Ascending aortic aneurysms may occur in those tension, recurrent coarctation, and aortic wall abnormal-
with BAV, and aneurysms may be present in the distal arch ities after repair. 1,2,6,24
2,11,19,20
and descending aorta. Untreated CoA may be
complicated by aortic dissection, heart failure, ruptured
cerebral aneurysm, or complications from hypertension. 1. In patients with CoA, both MRI and CT are can detect
Repair of CoA is performed by endovascular, open surgical, coexistent BAV, examine the full thoracic aorta for
coexistent aneurysm disease or arch abnormalities, gradients across the coarctation.11 For individuals with
4,25
and assist in treatment planning. TTE is also can native or recurrent CoA and appropriate anatomic
detect the gradients across the site of the coarctation characteristics, endovascular treatment with stenting is
and assess for recoarctation (recurrence of a significant typically performed.2,3,6,9-12,29 Open surgical repair of
coarct). After repair of CoA, complications may occur, CoA may include subclavian flap aortoplasty, resection
including recoarctation, aortic aneurysm, pseudoa- and end-to-end anastomosis, interposition grafting, or
neurysm, and aortic dissection.2,3,11,12,26 Arch and bypass grafting, with the choice of procedure informed
descending aortic complications are better visualized by patient- and anatomic-specific characteristics.5,11 In
by MRI or CT than TTE. The optimal imaging frequency adults who have undergone a previous open surgical
after repair of CoA is not well established and is best CoA repair and develop recoarctation, aneurysm, or
individualized based on the type of repair, physical pseudoaneurysm, an endovascular approach (assuming
examination findings, and previous imaging findings.27 there is adequate iliofemoral access and absence of
After establishing stable aortic imaging after CoA involvement of the supra-aortic trunks) avoids the need
repair, surveillance imaging is often obtained every 3 for reoperation.2,3,9,12,29
20,28-30 6,8
to 5 years. Recoarctation occurs in about 10% 4. Patients with CoA are at risk for complications of hy-
after surgical repair and about 8% after balloon dila- pertension, including heart failure, stroke, coronary
tion.21 After endovascular repair of CoA, MRI or CT can artery disease, and aortic complications, so hyperten-
evaluate for complications, recoarctation, or sion should be assessed and in accordance with current
endoleaks.2,3,11 guidelines.13 Multiple studies have shown that persis-
2. Patients with a significant CoA typically have hyper- tent hypertension is common after CoA
tension in the upper extremities and a reduction in BP correction.3,6,7,23,24 Ambulatory BP monitoring and
in the lower extremities. The location of the CoA will exercise testing may be useful in the evaluation and
inform any BP differential between the left and right treatment of hypertension in patients with native CoA
arms. Physical examination may reveal a delay in and after repair.3,22,24
timing and a decreased amplitude of the femoral pulse. 5. Screening studies suggest that adults with CoA have an
After CoA repair, recurrent coarctation may occur. 10% prevalence of intracranial aneurysms (compared
Obtaining the BP in the upper and lower extremities with a prevalence of 2% in the normal adult popula-
assesses for native and recurrent coarctation. tion), with the greatest risk among older adults and
3. CoA presents with upper extremity hypertension, lower those with hypertension.14-16,18 Cost-effective analysis
extremity hypoperfusion, and imaging confirmation of supports screening for intracranial aneurysms in adults
narrowing of the aorta that may include collateral for- with CoA, but preferred screening strategies remain
mation.2,3,6,11 Significant native or recoarctation has unknown.17 Because many of the intracranial aneu-
been variably defined, but commonly used criteria are rysms detected by screening will be very small and not
listed in Table 37. 7,11 The presence of left ventricular require treatment, shared decision-making about
hypertrophy is an important marker of disease. 28 In screening may be informed by age, risk factors, and
addition to abnormal aortic gradients, anatomic evi- anticoagulation considerations. 18,30
dence for CoA is necessary and is well characterized by
MRI or CT. Adult congenital guidelines have reported
9.4.2. Other Arch Abnormalities
the best evidence to proceed with intervention to cor-
rect CoA, including hypertension, BP differential be- 9.4.2.1. Aberrant Subclavian Artery, Kommerell’s
tween upper and lower extremities, and TTE-derived Diverticulum
Recommendations for Aberrant Subclavian Artery, Kommerell’s Diverticulum
1. In patients discovered to have an ASCA in the absence of thoracic aortic imaging, dedicated imaging to
2a C-LD assess for TAA is reasonable.1,2
2. In patients with Kommerell’s diverticulum, depending on patient anatomy and comorbidities, repair may
2b C-LD be reasonable when the diverticulum orifice is >3.0 cm, the combined diameter of the diverticulum and
adjacent descending aorta is >5.0 cm, or both (Figure 27). 3
Synopsis
F I G U R E 2 7 Measurements of Kommerell’s Diverticulum
Anomalies of the aortic arch are usually detected
incidentally on a CT of the chest or neck ordered for
other reasons. An ASCA arises as the fourth branch
from the aorta, distal to the left subclavian artery (or
right subclavian artery in the case of a right-sided
aortic arch). It courses through the posterior medias-
tinum behind the esophagus in its path to perfuse the
arm and can cause a vascular ring around the trachea
and esophagus that results in dysphagia, respiratory
symptoms, or recurrent laryngeal nerve palsy. Kom-
merell’s diverticulum is a persistent remnant of the
fourth primitive dorsal aortic arch because of failed
regression 3 and may be present in 20% to 60% of pa-
tients with an aberrant right or left subclavian artery.
The risk of rupture or dissection of a Kommerell’s
diverticulum has been reported to be as high as 50% in
case series, although high-quality data on the natural
history are very limited. The 2020 SVS clinical practice
guidelines recommend surgical intervention for Kom-
merell’s diverticulum when the diverticulum orifice is
>3.0 cm, the combined diameter of the diverticulum
and adjacent descending aorta is >5.0 cm, or both. 4
Successful repair has been described using open,
endovascular, and hybrid approaches depending on
patient anatomy and comorbidities.3,5
Two diameter measurements should be obtained using cross-sectional
Recommendation-Specific Supportive Text imaging: the diverticulum orifice (radially and longitudinally at the
aortic wall) and the combined diameter of the diverticulum and adja-
1. Variant aortic arch anatomy has been found to be cent descending thoracic aorta (measured from the apex of the
significantly associated with TAA in several single- diverticulum to the opposite aortic wall). ARSA indicates aberrant right
subclavian artery; LCA, left common carotid artery; LSA, left subclavian
center retrospective observational series, 1 with 33%
artery; and RCA, right common carotid artery. Adapted from Erben
of patients with right-sided aortic arch having et al,8 Copyright 2020, with permission from Elsevier, Inc., and the
concomitant TAA. 2 Left-sided aortic arch with aber- Society for Vascular Surgery.
rant right subclavian artery was also significantly
associated with TAD but only occurred in 2% to 8%
of those patients. 1 Consequently, if the imaging sectional imaging: the diverticulum orifice (radially
study that detected the ASCA did not include imag- and longitudinally at the aortic wall) and the com-
ing of the thoracic aorta, then a dedicated CT or MRI bined diameter of the diverticulum and adjacent
to evaluate for an associated aortic aneurysm is descending thoracic aorta (measured from the tip of
reasonable. the diverticulum to the opposite aortic wall6). Repair
2. Case series have reported rupture and/or dissection of of Kommerell’s diverticulum has been suggested
Kommerell’s diverticulum for diverticula ranging from when the orifice diameter is >3 cm or the combined
4.0 cm to 10 cm (mean size, 5.0 cm).3 The measure- diameter of the diverticulum and adjacent descending
ment of the Kommerell’s diverticulum may be diffi- thoracic aorta is >5.0 cm.3,4,7
cult, and various strategies to standardize measures
have been proposed.3 Based on CT, 2 diameter mea-
9.4.2.2. Aberrant Left Vertebral Artery Origin
surements should be obtained (Figure 27) using cross-
Recommendation for Aberrant Left Vertebral Artery Origin
1. In patients with an aberrant left vertebral artery origin arising directly from the thoracic aorta who require
2a C-EO aortic repair involving reconstruction or coverage of the vertebral artery origin, revascularization of the
vertebral artery is reasonable.
Synopsis open bypass or transposition technique can be accom-

The most common anatomic variant for the left verte- plished with good outcomes. 3,4
bral artery is arising directly from the aortic arch; 6% of
adults have a left vertebral artery that arises from the arch
1,2
between the left carotid and left subclavian arteries, 1. In patients undergoing elective TEVAR with planned left
rather than of a branch of the left subclavian artery. subclavian artery coverage, preoperative revasculariza-
There is a paucity of data on the management of the left tion of the left subclavian artery has been shown to
vertebral artery arising from the aortic arch in patients decrease the risk of stroke and SCI,5-8 presumably by
undergoing thoracic aortic repair. For patients undergoing maintaining perfusion through the posterior circulation
elective open surgical partial or total arch repair or un- via the left vertebral artery. In a small series of 9 patients
dergoing TEVAR for TAA or dissection, revascularization with an aberrant left vertebral artery origin undergoing
of the left subclavian artery is recommended to preserve open aortic arch replacement, no neurologic complica-
left vertebral artery perfusion and reduce the risk of tions were reported among patients who first underwent
symptomatic vertebrobasilar insufficiency, SCI, and revascularization of the left vertebral artery. 4
3
stroke. This may be particularly important in patients
9.4.2.3. Bovine Arch (Common Innominate
with a dominant left vertebral artery or a nonintact circle
and Left Carotid Artery)
of Willis. Vertebral artery revascularization via either an
Recommendation for Bovine Arch (Common Innominate and Left Carotid Artery)
1. In patients with bovine arch (common innominate and left carotid artery), imaging to assess for TAA may
2b C-LD be reasonable.1-3
F I G U R E 2 8 Normal and Bovine Aortic Arch Configurations
(A) Type I aortic arch: The normal aortic arch configuration. (B) Type II-A aortic arch: The left common carotid artery originates from the innominate artery. (C) Type II-B
aortic arch: The innominate and left common carotid arteries share a common origin. LCA indicates left common carotid artery; LSA, left subclavian artery; LVA, left
vertebral artery; RCA, right common carotid artery; RSA, right subclavian artery; and RVA, right vertebral artery. Adapted from Layton et al.5 Copyright 2006, American
Society of Neuroradiology. Used with permission from Mayo Foundation for Medical Education and Research, all rights reserved.

The most common anatomic pattern of great vessel
1. A bovine aortic arch appears to be a marker for TAD and
origin, occurring in approximately 70% of adults, is a type
more rapid aortic expansion. 1 Among patients with
I arch, in which the 3 great vessels originate directly from
TAD, the prevalence of a bovine arch was 26.3%,
the aorta. 4 Bovine arch variants are the most common
compared with 16.4% in controls (P<0.001). Moreover,
arch anomalies, and 2 types are described: In type II-A,
among patients with TAA, the annual aortic growth rate
found in 9% of the population, the left common carotid
was 0.29 cm/y among those with a bovine arch versus
artery arises directly from the innominate artery
0.09 cm/y among those with normal arch anatomy. A
(Figure 28); in type II-B, found in 13% of the population,
recent meta-analysis found that the proportion of TAD
the innominate and left common carotid arteries arise
among patients with bovine arch was 41.5%, compared
from a common origin (Figure 28).5 The term “bovine
with 34.0% among patients with standard arch config-
arch” is a misnomer, because the arch vasculature in
uration.3 If aortic dilation or aneurysm is found on
cattle has a single, large brachiocephalic vessel that sub-
imaging, subsequent surveillance imaging may be
sequently trifurcates into 2 subclavian arteries and a
obtained.
bicarotid trunk.5 Others have referred to the bovine aortic
arch pattern as an aortic arch with a common origin of the
innominate and left carotid artery. 9.5. Tumors
Some authors have suggested that a bovine arch in- Tumors of the thoracic aorta are usually secondary,
creases the risk of aortic dissection, but the data are resulting from contiguous or metastatic spread of primary
limited. 2,6 Among patients with acute type A aortic malignancies, especially lung and esophageal. 1,2 Primary
dissection, a bovine arch was highly predictive of an arch malignant tumors of the aorta, which are extremely rare,
tear (OR, 5.9; 95% CI, 2.89–12.04; P<0.001) and increased are most often primary sarcomas that protrude into the
perioperative stroke (OR, 2.69; 95% CI, 1.2–6.0; P¼0.016) lumen but leave the aortic wall intact. Aortic sarcomas are
based on multivariable analysis, although it was not aggressive tumors with a propensity for arterial emboli-
associated with worse long-term survival. 7 zation, disseminated metastases, and rapid clinical
deterioration,3,4 usually with limited survival after initial postmortem. Combined therapy with surgery (resection
diagnosis. 5,6 Tumors of the thoracoabdominal aorta may and reconstruction of the segment of aorta containing the
exhibit nonspecific symptoms. On imaging, aortic tumors neoplasm) and chemoradiotherapy provide the best sur-
are often initially mistaken for atherosclerosis or aneu- vival results, although the overall prognosis remains
rysmal disease 7 (although PET imaging may suggest tu- poor.
mor metabolic activity over metabolically quiescent
atherosclerosis), so the diagnosis is often made by histo- 10. PHYSICAL ACTIVITY AND QUALITY OF LIFE
logic examination of embolic debris or surgical speci-
mens8-10 ; in some cases, the diagnosis is made
Recommendations for Physical Activity and Quality of Life
1. For patients with significant aortic disease, education and guidance should be provided about avoiding
1 C-EO intense isometric exercises (eg, heavy weightlifting or activities requiring the Valsalva maneuver), burst
exertion and activities, and collision sports. 1,2
2. For patients who have undergone surgery for aortic aneurysm or dissection, postoperative cardiac
1 C-EO rehabilitation is recommended. 3,4
3. In patients with thoracic or abdominal aortic aneurysms whose BP is adequately controlled, it is

2a C-LD reasonable to encourage 30 to 60 minutes of mild-to-moderate intensity aerobic activity at least 3 to 4
days per week.5,6
4. For patients with clinically significant aortic disease, it is reasonable to screen for anxiety, depression,
2a C-LD and posttraumatic stress disorder and, when indicated, provide resources for support 7,8 ; it is also
reasonable to provide education and resources to minimize patients’ concerns, support optimal decision-
making, and enhance quality of life. 5,9-11
Synopsis Patients with aortic aneurysms, who have adequate BP

As surgical outcomes for aortic disease improve, a focus control, may have improvements in overall cardiovascular
on patient-reported health-related quality of life (HRQOL) health when undertaking moderate intensity aerobic ac-
outcomes is becoming increasingly important, 10 because tivity at least 3 to 4 days per week, 30 to 60 minutes per
patients have become increasingly concerned about session.17-19 Although resistance training may be beneficial
HRQOL issues such as returning to work, pain manage- to patients with cardiovascular disease, it increases cen-
ment, risk of infection, activity recommendations and re- tral aortic BP and, therefore, benefits for those with aortic
strictions, and neurologic complications. The most aneurysm are less well understood because, theoretically,
common measures of HRQOL are generic patient-reported increases in BP could contribute to subsequent aortic
outcome measures (eg, SF-36), although validated growth, complications, or both. Further longitudinal study
aneurysm-specific measures have been developed.7,12,13 is warranted. 20-22
In patients with Marfan syndrome in the GenTAC reg-
istry, HRQOL was slightly below the population norm.
Better HRQOL was independently associated with socio- 1. In patients with aortic disease, limited data are avail-
economic factors (eg, private insurance, active employ- able to guide recommendations regarding the forms of
ment) but not factors related to disease severity or exercise that are safe and promote cardiovascular
comorbidities. 14,15 Although aneurysms are usually health versus those that pose an acute or long-term risk
asymptomatic before diagnosis, surgical aortic repair is of aortic growth or rupture. But evidence exists
associated with an initial deterioration in HRQOL at 3 regarding the physiologic benefits of exercise and the
months, including decreased physical, cognitive, and so- hemodynamic consequences of various form of exercise
cial function that generally returns to preoperative levels and exertion in case series and relevant animal models.
after 6 to 12 months. 11 Standardized reporting of preop- There has been a uniform consensus among numerous
erative and postoperative HRQOL measures is needed to expert committees on aortic disease that it is wise to
guide further improvements in interventional strategies avoid intense isometric exertion or exercises that
and improve the overall patient experience. 16 require the Valsalva maneuver, given that heavy lifting
with Valsalva can produce acute increases in SBP to moderate—but not strenuous—aerobic exercise pro-
>300 mm Hg. There is also a consensus that light tected the structural integrity of the aortic wall, as
weightlifting and low-intensity aerobic exercise are evidenced by reduced elastin fragmentation and
safe and likely improve both physical and mental reduced expression of matrix metalloproteinases 2 and
health. No uniform consensus exists about the safety 9 within the aortic wall, compared with sedentary
of intermediate-level static and aerobic exercise. Rec- controls.39
ommendations for exercise intensity are best individ- 4. Depression and anxiety often occur in patients with
ualized, informed by multiple factors that include aortic disease, regardless of surgical status. Post-
underlying aortic pathology, aortic diameter and ASI, traumatic stress disorder after dissection is a particular
aortic growth rate, age, family history, and any other risk. 8 Screening patients and providing resources for
high-risk features (eg, uncontrolled hypertension). assistance may prevent mental health issues from
Ongoing investigation is needed to better define the becoming more severe and lead to an increased
levels of resistance activities that would be considered HRQOL.9,40 The SF-36 is a common tool for assessing
low-risk for adverse aortic events, favoring greater mental health for these patients 7,11,12,41 but may not
exercise restrictions among patients at higher risk of cover all patient concerns, such as activity restriction,
dissection.17,23-26,27 family life, and losing ability to earn income. 16 More
2. Although data are limited, cardiac rehabilitation has studies are needed with both pre- and postoperative
been shown to be useful and safe for patients after HRQOL data to improve shared decision-making and
aortic surgery.4,5,27,28 A randomized trial of exercise- patient outcomes.12,13,41 Exercise may decrease
based cardiac rehabilitation in patients who have un- depression.9 Education before procedures helps most
dergone surgery for type A aortic dissection showed patients feel more satisfied with their procedures 16 and
improved peak oxygen uptake, maximal workload, and improve postoperative HRQOL. 41 Patients and clini-
3
HRQOL. Fear of a repeat cardiac event can cause pa- cians can define surgery success differently, showing
tients who are post–aortic dissection to decrease or the importance of discussing expectations and risks.
stop exercise and sexual activity, but mild-to-
moderate intensity exercise may be cardioprotective.
Because of deconditioning, patients may be unable to 11. COST AND VALUE CONSIDERATIONS
exercise initially at the recommended level. 27 An in-
tensity of 3 to 5 metabolic equivalents of task is rec- Although assessment of cost and value in development of
ommended, while avoiding strenuous lifting, lifting to guidelines is of growing importance, studies are limited
the point of exhaustion, or other activities that entail on the cost-effectiveness of aortic disease treatment and
maximal exertion. 6,29 In a retrospective study, patients lack standard methods for comparison.1
with small AAA went through a modified cardiac Screening for AAA among men $65 years of age has
rehabilitation program before surgery, and the rate of been shown to be cost-effective, 2,3 although data for
aortic growth was slower in the rehabilitation group. 28 screening women are less clear. Women have a lower
3. High-intensity athletic training in 1 study has been incidence of AAA but higher risk of rupture and longer life
shown to be an independent predictor of aortic growth, expectancy, so incremental cost-effectiveness is similar
although these data were limited to the aortic root and to men and may justify screening, especially in those with
did not include AAA. 30 In a recent study in 442 athletes a history of smoking.4
of mean age 61 years, aortic root enlargement by z- In patients with AAA, studies comparing EVAR to open
score was present in 24% of participants and, after surgical repair generally show lower initial costs for EVAR
multivariate analysis, elite competitor status was based on shorter hospital stays; however, ongoing ex-
found to be an independent predictor of aortic penses for EVAR surveillance and reinterventions may
growth. 31 Less is known about the potential effects of minimize long-term cost advantages after 2 to 5 years. 5-9
mild-to-moderate intensity aerobic activity on aortic In addition, significant variability in costs across organi-
growth, but it is known to improve overall cardiovas- zations and countries, and changing efficiencies in tech-
cular health, including among patients with TAA 32-34 niques, makes it difficult to make recommendations on
and AAA.20,35,36 A recent meta-analysis suggests that preferred interventional approaches based primarily on
that higher physical activity is associated with a relative costs.6,10,11
37
reduced risk of AAA. In 1 study of a mouse model of Findings are mixed but similar for descending TAA,
Marfan syndrome, rates of aortic root growth were with trends toward lower initial hospital costs with
significant slower in mice that exercised daily on a TEVAR compared with open surgery stemming from
treadmill compared with sedentary mice.38 In another shorter length of stay, but the long-term results are more
study of mice with Marfan syndrome, both mild and neutral.12-14
Few data examine the cost-effectiveness of manage- 12.2. Genetic and Nongenetic Factors
ment strategies of TAA. For the management of AAS, the Various genes have been associated with and linked to
costs are not easily modifiable. However, for management TAA and dissection. Consequently, genetic testing can
of chronic TAD, patients often see a host of specialists, identify pathogenic mutations in specific genes that in-
including both cardiologists and surgeons, have follow-up crease a patient’s risk of aneurysm, dissection, or both
visits with specialists in both the community and at ter- and may inform the optimal timing of aortic repair. As the
tiary or quaternary centers, or both. Moreover, diagnostic prevalence of genetic testing increases, the discovery of
imaging is often duplicated because of differences in more genes will help in the earlier diagnosis of asymp-
imaging protocols or quality, or simply because images tomatic nonsyndromic TAA. In addition to the contribu-
are not readily transferrable. Consequently, there are tion of genetic variants, environmental factors and
likely opportunities for significant cost savings if redun- lifestyle habits may contribute to aortic aneurysm for-
dant clinician visits and imaging could be reduced mation. Further research on these factors may provide
through common protocols, common imaging platforms, evidence to guide lifestyle modifications that could
and coordinated care.15 reduce a patient’s lifelong aortic risk. Recent evidence
suggests that fluoroquinolone use is associated with an
12. EVIDENCE GAPS AND FUTURE DIRECTIONS increased risk of aortic aneurysm and dissection, but the
pathways through which this effect is mediated are un-
Most of the current recommendations for patients with known. Future research investigating the potentially
aortic disease are based on expert opinion and data from protective or harmful effects of other pharmacologic
observational studies, large registries, and prospective agents on aortic health might further elucidate the path-
studies, but few are from randomized clinical trials. More ophysiology of aortic disease.1-10
data are needed from basic science studies and RCTs to
guide prevention, early diagnosis, and advanced treat- 12.3. Biomechanics of the Aorta
ment for aortic disease. In the future, precision medicine Emerging evidence suggests that aortic diameter alone is
and patient-centered approaches will enable clinicians to an insufficient predictor of risk for aortic dissection. Un-
develop care plans to optimize outcomes for each patient. derstanding the distribution of biomechanical wall stress
Future research should include diverse populations and in the various anatomic locations of the aorta, as well as
examine race, ethnicity, and sex differences to ensure potential contributions of hemodynamic flow distur-
that all patient groups are represented and that questions bances such as those from aortic valve stenosis or regur-
pertinent to their aortic health are answered. gitation, or even from a well-functioning BAV, may
improve risk stratification strategies and, in turn, patient
12.1. Biomarker Studies outcomes, filling a knowledge gap on wall stress distri-
Although interest in using circulating biomarkers for risk bution in patients with aortic aneurysms. 1-4
stratification of patients with aortopathy has increased,
biomarker expression has not been clearly associated with 12.4. Sex, Race, and Ethnicity
relevant clinical aortic events. Most studies have focused Conflicting data exist in the literature on the association
on protein-based biomarkers and noncoding RNAs in pa- between sex and outcome in patients with aortic disease.
tients with bicuspid aortopathy. These emerging bio- Studies have shown different rates of aortic aneurysm
markers and other better, early-stage biomarkers, along growth and dissection risk in male versus female patients.
with advanced noninvasive imaging modalities, may help Nevertheless, the data are inconsistent, because some
us precisely identify the risk associated with adverse outcome studies indicate that sex affects prognosis,
outcomes in these patients. In addition, noncoding RNAs whereas others show no impact of sex. Clearly, further
such as microRNA are biological molecules whose research is needed to elucidate the impact of sex on the
expression can be modified through targeted mechanisms incidence and progression of aortic disease, the risk of
and present opportunities to identify new treatment op- aortic dissection, and the outcomes of intervention. Even
tions for patients with aortic disease. 1-7 In addition, more challenging is the fact that few studies have been
developing image-based cardiac and aortic markers published on racial and ethnic disparities among patients
derived from large-scale imaging studies with automated with aortic disease and those undergoing aortic inter-
machine learning–based analysis might provide a wealth vention. Moreover, it is unclear that all patients with
of information for guiding the optimal care of these aortic disease have equal access to skilled practitioners to
patients. care for them, so it is imperative that we seek ways to
actively minimize such health care disparities.1-17 Simi- still have untreated diseased aortic segments after surgi-
larly, efforts should be made to broaden clinical trials to cal aortic repair and who do not meet the surgical
represent the diverse populations that we treat; study threshold for intervention. Developing patient-centric
design, methodology, reporting, and implementation rehabilitation protocols and individualized exercise pro-
should be designed to be more inclusive. 18,19 grams for patients with aortic disease is an unmet need
that requires further study.1-4
12.5. Quality of Life in Patients With Aortic Disease
Baseline HRQOL assessment in patients with aortic dis- 12.8. Equitable Care and Training Opportunities
ease is lacking, and the few studies that have targeted Sociodemographic disparities can pose challenges to pa-
HRQOL have been conducted only in patients receiving tients and clinicians who seek and offer cardiovascular
endovascular or open aortic repair. The impact of phys- and aortic care. Market competition, a relatively modern
ical, mental, emotional, sexual, and professional status on phenomenon, and physician market concentration can
the psychosocial well-being, tolerance of medical thera- drive decision-making and subsequently affect optimal
pies, and recovery from aortic intervention has not been care. Providing optimal cardiovascular and aortic care will
well studied. The long-term effects on physical and depend on widespread regional quality improvement
mental HRQOL after aortic repair are unknown. In addi- projects to determine best practices, minimize variations
tion, evidence-based knowledge on studies targeting in areas where evidence-based medicine has finite
quality of life in patients with heritable TAA is narrow or benchmarks, and standardize patient selection and case
limited only to patients with Marfan syndrome; almost no management. Physician participation in these programs
studies have been performed in patients with Loeys-Dietz should be encouraged, and educational interventions and
syndrome or vascular Ehlers-Danlos syndrome, for training should be provided to disseminate knowledge
example. Furthermore, only scattered studies have and improve performance, which will help increase
examined strategies for boosting the psychological health awareness for patients and physicians in less-populated,
of patients with aortic disease and those undergoing underserved areas.1-3
aortic surgery. Aortic diseases require a lifetime of treat-
ment and surveillance, so research is needed on ways to PRESIDENTS AND STAFF
improve and sustain patient engagement, especially
among those who are disadvantaged or at a lower American College of Cardiology
educational level.1-8 Edward T.A. Fry, MD, FACC, President
Cathleen C. Gates, Chief Executive Officer
12.6. New Endovascular Technology Richard J. Kovacs, MD, MACC, Chief Medical Adviser
Advances in endovascular technology have dramati- MaryAnne Elma, MPH, Senior Director, Enterprise
cally impacted treatment strategies in patients with Content and Digital Strategy
aortic disease requiring intervention. Despite this sig- Grace D. Ronan, Team Leader, Clinical Policy Publications
nificant progress, current endovascular designs are Leah Patterson, Project Manager, Clinical Content
limited in their application because of the differing Development
hemodynamic and anatomic challenges presented by American College of Cardiology/American Heart Association
each segment of the aorta and individual differences Thomas S.D. Getchius, Director, Guideline Strategy and
in aortic anatomy. In addition, operator knowledge Operations
and experience, as well as methodical patient selec- Abdul R. Abdullah, MD, Director, Guideline Science and
tion, are important for obtaining optimal outcomes Methodology
from endovascular procedures. Continued evolution in American Heart Association
stent-graft design, focused on flexibility and durability, Donald M. Lloyd-Jones, MD, ScM, FAHA, President
improved vascular imaging technology, and advances Nancy Brown, Chief Executive Officer
in simulation training for operators, will likely further Mariell Jessup, MD, FAHA, Chief Science and Medical
reduce the risk of reinterventions and improve long- Officer
term outcomes.1-6 Radhika Rajgopal Singh, PhD, Senior Vice President,
Office of Science and Medicine
12.7. Optimal Exercise and Rehabilitation Protocols Johanna A. Sharp, MSN, RN, Science and Medicine
Very limited research has been conducted on optimal Advisor, Office of Science, Medicine and Health
exercise in patients with aortic disease. Moreover, no Jody Hundley, Production and Operations Manager,
specific rehabilitation strategies exist for patients who Scientific Publications, Office of Science Operations
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als in more than 230,000 patients. Ann Thorac Surg.
ture review. Arch Cardiol Mex. 2020;90:309–312.
3. Chou EL, Pettinger M, Haring B, et al. Association of 2021;112:726–735.
premature menopause with risk of abdominal aortic 2. DeFabio DC, DeFabio CJ. Exercise parameters for the
aneurysm in the Women’s Health Initiative. Ann Surg. 12.5. Quality of Life in Patients With Aortic chronic type B aortic dissection patient: a literature re-
Disease view and case report. Postgrad Med. 2021;133:217–222.
Published online November 4, 2020. https://doi.org/
10.1097/SLA.0000000000004581 3. Delsart P, Maldonado-Kauffmann P, Bic M, et al.
1. Archer S, Pinto A, Vuik S, et al. Surgery, complica-
4. Deery SE, Soden PA, Zettervall SL, et al. Sex dif- tions, and quality of life: a longitudinal cohort study Post aortic dissection: gap between activity recom-
ferences in mortality and morbidity following repair of exploring the role of psychosocial factors. Ann Surg. mendation and real life patients aerobic capacities. Int
intact abdominal aortic aneurysms. J Vasc Surg. 2019;270:95–101. J Cardiol. 2016;219:271–276.
2017;65:1006–1013.
2. Blakeslee-Carter J, Menon AJ, Novak Z, et al. As- 4. Fuglsang S, Heiberg J, Hjortdal VE, et al. Exercise-
5. Lo RC, Schermerhorn ML. Abdominal aortic aneu- sociation of mental health disorders and aortic based cardiac rehabilitation in surgically treated type-
rysms in women. J Vasc Surg. 2016;63:839–844. dissection. Ann Vasc Surg. 2021;77:217–225. A aortic dissection patients. Scand Cardiovasc J.
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6. Nicolini F, Vezzani A, Corradi F, et al. Gender dif- 3. Ghanta RK, Green SY, Price MD, et al. Midterm
ferences in outcomes after aortic aneurysm surgery survival and quality of life after extent II thor- 12.8. Equitable Care and Training Opportunities
should foster further research to improve screening acoabdominal aortic repair in Marfan syndrome. Ann
and prevention programmes. Eur J Prev Cardiol. Thorac Surg. 2016;101:1402–1409; discussion 1409. 1. Holscher CM, Weaver ML, Black JH 3rd, et al.
2018;25:32–41. Regional market competition is associated with aneu-
4. Luo ZR, Liao DS, Chen LW. Comparative analysis of
rysm diameter at the time of EVAR. Ann Vasc Surg.
7. Nienaber CA, Fattori R, Mehta RH, et al. Gender- postoperative sexual dysfunction and quality of life in
2021;70:190–196.
related differences in acute aortic dissection. Circula- type A aortic dissection patients of different ages.
tion. 2004;109:3014–3021. J Cardiothorac Surg. 2021;16:117. 2. Mullan CW, Mori M, Bin Mahmood SU, et al. Inci-
dence and characteristics of hospitalization for prox-
8. Olson SL, Wijesinha MA, Panthofer AM, et al. Eval- 5. Nilsson O, Stenman M, Letterstal A, et al.
imal aortic surgery for acute syndromes and for
uating growth patterns of abdominal aortic aneurysm A randomized clinical trial of an eHealth intervention
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diameter with serial computed tomography surveil- on anxiety in patients undergoing abdominal aortic
diothorac Surg. 2020;58:583–589.
lance. JAMA Surg. 2021;156:363–370. aneurysm surgery. Br J Surg. 2021;108:917–924.
9. Preventza O, Cekmecelioglu D, Chatterjee S, et al. 6. Smolock CJ, Xiang F, Roselli EE, et al. Health- 3. Zettervall SL, Buck DB, Soden PA, et al. Regional
Sex differences in ascending aortic and arch surgery: a related quality of life after extensive aortic replace- variation exists in patient selection and treatment of
propensity-matched comparison of 1153 pairs. Ann ment. Semin Thorac Cardiovasc Surg. 2021. S1043- abdominal aortic aneurysms. J Vasc Surg. 2016;64:921–
Thorac Surg. 2022;113:1153–1158. 0679:00318-X. 927.e1.
10. Rylski B, Georgieva N, Beyersdorf F, et al. Gender- 7. Thijssen CGE, Doze DE, Gokalp AL, et al. Male-fe-
related differences in patients with acute aortic male differences in quality of life and coping style in
dissection type A. J Thorac Cardiovasc Surg. 2021;162: patients with Marfan syndrome and hereditary thoracic KEY WORDS ACC/AHA Clinical Practice
528–535.e1. aortic diseases. J Genet Couns. 2020;29:1259–1269. Guidelines, abdominal aortic aneurysm, aortic
dissection, aortitis, aortopathy, bicuspid aortic
11. Spiliotopoulos K, Price MD, Amarasekara HS, et al. 8. Velvin G, Wilhelmsen JE, Johansen H, et al. Sys-
valve, blunt traumatic aortic injury, cardiac
Are outcomes of thoracoabdominal aortic aneurysm tematic review of quality of life in persons with he-
surgery, guidelines, endovascular aortic repair,
repair different in men versus women? A propensity- reditary thoracic aortic aneurysm and dissection
heritable thoracic aortic disease, intramural
matched comparison. J Thorac Cardiovasc Surg. diagnoses. Clin Genet. 2019;95:661–676.
hematoma, malperfusion syndrome, marfan
2017;154:1203–1214.e1206.
12.6. New Endovascular Technology syndrome, loeys-dietz syndrome, penetrating
12. Tomee SM, Lijftogt N, Vahl A, et al. A registry- atherosclerotic ulcer, thoracic aortic aneurysm,
based rationale for discrete intervention thresholds for 1. Czerny M, Rylski B, Morlock J, et al. Orthotopic thoracoabdominal aortic aneurysm, thoracic
open and endovascular elective abdominal aortic branched endovascular aortic arch repair in patients endovascular aortic repair, vascular surgery
-, 2022:-–-
JACC VOL.
APPENDIX 1. AUTHOR RELATIONSHIPS WITH INDUSTRY AND OTHER ENTITIES (RELEVANT)—2022 ACC/AHA GUIDELINE FOR THE DIAGNOSIS
AND MANAGEMENT OF AORTIC DISEASE
-, NO. -, 2022
Ownership/ Institutional,
Partnership/ Personal Organizational, or Expert
Committee Member Employment Consultant Speakers Bureau Principal Research Other Financial Benefit Witness
Eric M. Isselbacher (Chair) Massachusetts General Hospital—Director, None None None None None None
Healthcare Transformation Lab; Co-
Director, MGH Thoracic Aortic Center;
Harvard Medical School—Associate
Professor of Medicine
James Hamilton Black III (Vice Johns Hopkins Medicine—Professor of None None None None None None
Chair) Surgery
Ourania Preventza (Vice Chair) Baylor College of Medicine; Baylor n Terumo Aortic (Bolton Medical) None None None None None
St. Luke’s Medical Center—Professor n W.L. Gore & Associates
of Surgery, Division of Cardiothoracic
Surgery
John G. Augoustides University of Pennsylvania—Professor, None None None None None None
Department of Anesthesiology and
Critical Care
Adam W. Beck University of Alabama at Birmingham— n Cook Medical None None n Cook Medical None None
Professor and Director of Vascular n n
Cryolife Medtronic
Surgery and Endovascular Therapy,
n Endologix n Terumo Aortic
Department of Surgery
n Philips (Bolton Medical)
n W.L. Gore &
n Terumo Aortic (Bolton Medical)
Associates
Michael A. Bolen Cleveland Clinic, Main Campus—Associate None None None None None None
Professor of Radiology, Division of
Radiology
Alan C. Braverman Washington University School of None None None None None None
Medicine—Alumni Endowed Professor in
Cardiovascular Diseases; Director, Marfan
Syndrome and Aortopathy
Clinic Cardiovascular Division,
Department of Medicine
Bruce E. Bray University of Utah—Professor, Department None None None None None None
2022 ACC/AHA Aortic Disease Guideline

of Internal Medicine and Biomedical
Informatics
Maya M. Brown-Zimmerman Patient advocate None None None None None None
Edward P. Chen Duke University Medical Center—Professor None None None n Bolton Medical* n Bolton Medical† None
and Division Chief,
Division of Cardiovascular and Thoracic Surgery
Isselbacher et al
Continued on the next page
165
166
Isselbacher et al
APPEDENDIX 1. CONTINUED
Tyrone J. Collins Ochsner Medical Center—Section Head, n InspireMD None None None n W.L. Gore & None
Interventional Cardiology, Co-Director, Associates†
Cardiac Catheterization Laboratory,
Department of Interventional Cardiology
Abe DeAnda Jr UTMB-Galveston—Professor and Chief, None None None None None None
Division of Cardiovascular and Thoracic
Surgery,
Division of Surgery
Christina L. Fanola University of Minnesota—Assistant n Janssen Pharmaceuticals None None None None None
Professor, Department of Cardiovascular
Medicine
Leonard N. Girardi Weill Cornell Medicine—Professor and None None None None None None
Chairman,
Department of Cardiothoracic Surgery
Caitlin W. Hicks Johns Hopkins University School of None None None None None None
Medicine—Associate Professor of Surgery,
Division of Surgery
Dawn S. Hui University of Texas Health San Antonio— None None None n Astellas Pharma* None None
Associate Professor, Department of
Cardiothoracic Surgery
William Schuyler Jones Duke University—Associate Professor of n Bayer‡ None None n Boehringer None None
Medicine & Director of Cardiac n Ingelheim
Janssen Pharmaceuticals‡
Catheterization Laboratory, Division of n Bristol-Myers
Medicine/Cardiology Squibb
Vidyasagar Kalahasti Cleveland Clinic—Assistant Professor, None None None None None None
Cleveland Clinic Lerner College of Medicine
of the Case Western Reserve University;
Director, Marfan Syndrome & Connective
Tissue Disorder Clinic,
Aortic Center, Heart, Vascular and Thoracic
Institute
Karen M. Kim University of Michigan—Assistant None None None None None None
Professor,
Department of Cardiac Surgery
JACC VOL.
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-, 2022:-–-
JACC VOL.
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Dianna M. Milewicz University of Texas Health Science Center None None None None None None
at Houston—President George H.W. Bush
Chair of Cardiovascular Medicine Vice Chair,
Department of Internal Medicine Director,
Division of Medical Genetics Director,
Division of Internal Medicine
Gustavo S. Oderich The University of Texas Health Science n Cook Medical None None n Cook Medical‡ n Centerline Biomed- None
Center at Houston, McGovern Medical n n ical, Advisory Board
GE Healthcare GE Healthcare‡
School—Professor of Surgery and Chief of Member*
n W.L. Gore & Associates n W.L. Gore &
Vascular and Endovascular Surgery, n Cook Medical†
Director of Aortic Center, Associates‡
n Philips, Advisory
Department of Cardio-Thoracic and Vascular
Surgery Board Member
n W.L. Gore &
Associates†
Laura Ogbechie Baylor College of Medicine—Nurse None None None None None None
Practitioner,
Department of Cardiothoracic Surgery
Susan B. Promes Penn State Health Hershey Medical None None None None None None
Center—Professor and Chair, Department of
Emergency Medicine
Elsie Gyang Ross Stanford University School of Medicine— None None None None None None
Assistant Professor,
Department of Surgery and Medicine
Marc L. Schermerhorn Beth Israel Deaconess Medical Center— None None None None n Medtronic, None
George H. A. Clowes Jr. Professor of Scientific Advisory
Surgery, Chief, Division of Vascular and Board Member*
Endovascular Surgery, n Philips, Scientific
Department of Surgery Advisory Board
Member*
Sabrina Singleton Times§ American Heart Association/American None None None None None None
College of Cardiology Guideline Advisor

Isselbacher et al
167
168
Isselbacher et al
Elaine E. Tseng University of California San Francisco; San None None None None n Cryolife†‡ None
Francisco VA Medical Center—Professor of
Surgery University of California San
Francisco; Chief of Cardiothoracic Surgery,
San Francisco VA Department of Surgery
Grace J. Wang University of Pennsylvania Hospital— None None None None None None
Associate Professor of Surgery, Division of
Vascular Surgery and Endovascular Therapy
Y. Joseph Woo Stanford University—Chair, Division of None None None None None None
Cardiothoracic Surgery
This table represents the relationships of committee members with industry and other entities that were determined to be relevant to this document. These relationships were reviewed and updated in conjunction with all meetings and/or conference calls
of the writing committee during the document development process. The table does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents
ownership of $5% of the voting stock or share of the business entity, or ownership of $$5,000 of the fair market value of the business entity; or if funds received by the person from the business entity exceed 5% of the person’s gross income for the
previous year. Relationships that exist with no financial benefit are also included for the purpose of transparency.
Relationships in this table are modest unless otherwise noted. According to the ACC/AHA, a person has a relevant relationship IF: a) the relationship or interest relates to the same or similar subject matter, intellectual property or asset, topic, or issue
addressed in the document; or b) the company/entity (with whom the relationship exists) makes a drug, drug class, or device addressed in the document or makes a competing drug or device addressed in the document; or c) the person or a member of the
person’s household, has a reasonable potential for financial, professional or other personal gain or loss as a result of the issues/content addressed in the document.
*No financial benefit.
†This disclosure was entered under the Clinical Trial Enroller category in the ACC’s disclosure system. To appear in this category, the author acknowledges that there is no direct or institutional relationship with the trial sponsor as defined in the (ACCF or
AHA/ACC) Disclosure Policy for Writing Committees.
‡Significant relationship.
§Sabrina Singleton Times is an AHA/ACC joint staff member and acts as the Guideline Advisor for the “2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease.” No relevant relationships to report. Nonvoting author on measures and
not included/counted in the RWI balance for this committee.
ACC indicates American College of Cardiology; ACCF, American College of Cardiology Foundation; AHA, American Heart Association; MGH, Massachusetts General Hospital; RWI, relationships with industry and other entities; UTMB, University of Texas
Medical Branch; and VA, Veterans Affairs.
JACC VOL.
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JACC VOL.
APPENDIX 2. REVIEWER RELATIONSHIPS WITH INDUSTRY AND OTHER ENTITIES (COMPREHENSIVE)—2022 ACC/AHA GUIDELINE FOR THE
DIAGNOSIS AND MANAGEMENT OF AORTIC DISEASE
-, NO. -, 2022
Speakers Partnership/ Personal Organizational, or Other Expert
Reviewer Employment Consultant Bureau Principal Research Financial Benefit Witness
David P. Faxon (Peer Review Harvard University; None None n REVA Medical n Boston Scientific None None
Committee Chair) Brigham and Women’s (DSMB)
Hospital n CSL Behring (DSMB)
n Medtronic (DSMB)*
Gilbert R. Upchurch Jr University of Florida None None n Antyllus* n NIH, PI* n Bolton† None
(Peer Review College of Medicine n Cook†
Committee Vice Chair)
n Gore†
n Medtronic†
Aaron W. Aday Vanderbilt University n OptumCare‡ None None n Janssen Pharmaceuticals† None
Medical Center n n
TransThera Sciences University of Manitoba†
Ali Azizzadeh Cedars Sinai None None None None n Gore† None
Michael Boisen University of Pittsburgh None None None None None None
(representing SCA)
Mohammad H. Eslami University of Pittsburgh None None None None None None
(representing SVS)
Beau Hawkins (representing The University of Oklahoma n Baim Institute for None None None n Behring† None
SVM) College of Medicine Clinical Research n Boston Scientific†
n Hemostemix†
n NIH†
Christopher M. Kramer University of Virginia n Bristol Myers None None n NHLBI‡ n Cytokinetics† None
Squibb‡ n NIBIB‡ n MyoKardia†
n Cytokinetics
n Eli Lilly
n Xencor
Jessica G. Y. Luc University of British None None None None None None
Columbia

Thomas E. MacGillivary Houston Methodist None None None None n Xylocor† None
(representing STS)
Isselbacher et al
169
170
Isselbacher et al
S. Christopher Malaisrie Northwestern University n Artivion n Edwards None n Artivion‡ n Artivion† None
(representing AATS) n Lifesciences‡ n n
Medtronic Atricure Edwards Lifesciences†
n Terumo n Edwards Lifesciences‡ n Medtronic†
n Terumo‡
Kathryn Osteen Baylor University Louise None None None None n AHA* None
Herrington School of n Congenital Heart Public
Nursing
Health Consortium*
n Sigma Theta Tau-Eta
Gamma Chapter*
n The Children’s Heart
Foundation*
Himanshu J. Patel University of Michigan n Edwards None None None n Edwards Lifesciences† None
Health Lifesciences n Gore†
n Gore n Medtronic†
n Medtronic‡ n Nexus†
n Terumo
Parag J. Patel (representing Medical College of None None None None n AHA* None
SIR) Wisconsin n SIR*
Wanda M. Popescu Yale School of Medicine None None None None n Ambu, Advisory Board None
(representing SCA) Member
Evelio Rodriguez Ascension Tennessee Saint n Abbott‡ n Abbott‡ None n Abbott n Abbott† None
Thomas Hospital n n n n
Edwards Edwards Atricure Atricure†
Lifesciences‡ Lifesciences‡ n Boston Scientific n Boston Scientific†
n Philips‡ n Claret n Edwards Lifesciences†
n Direct Flow n Medtronic†
n Edwards Lifesciences‡
n Medtronic
Rebecca Sorber Johns Hopkins University None None None None None None
School of Medicine
Philip S. Tsao VA Palo Alto Health Care None None None None None None
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System; Stanford
University School of
Medicine
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JACC VOL.
-, NO. -, 2022
Annabelle Santos Rush College of Medicine n Bristol Myers None None n NIH‡ n Apple* None
Volgman Squibb, DCICDP n Novartis†
n Janssen
Pharmaceuticals
n Merck
n Pfizer
n Sanofi‡
This table represents all reviewers’ relationships with industry and other entities that were reported at the time of peer review, including those not deemed to be relevant to this document, at the time this document was under review. The table does not
necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of 5% of the voting stock or share of the business entity, or ownership of $5000 of
the fair market value of the business entity; or if funds received by the person from the business entity exceed 5% of the person’s gross income for the previous year. Relationships that exist with no financial benefit are also included for the purpose of
transparency. Relationships in this table are modest unless otherwise noted. Names are listed in alphabetical order within each category of review. Please refer to https://www.acc.org/guidelines/about-guidelines-and-clinical-documents/ relationships-
with-industry-policy for definitions of disclosure categories or additional information about the ACC/AHA Disclosure Policy for Writing Committees.
*No financial benefit.
†This disclosure was entered under the Clinical Trial Enroller category in the ACC’s disclosure system. To appear in this category, the author acknowledges that there is no direct or institutional relationship with the trial sponsor as defined in the (ACCF or
AHA/ACC) Disclosure Policy for Writing Committees.
‡Significant relationship.
AATS indicates American Association for Thoracic Surgery; ACC, American College of Cardiology; ACCF, American College of Cardiology Foundation; AHA, American Heart Association; DCICDP, Diverse Clinical Investigator Career Development Program;
DSMB, data and safety monitoring board; NIH, National Institutes of Health; NHLBI, National Heart, Lung, and Blood Institute; NIBIB, National Institute of Biomedical Imaging and Bioengineering; PRC, Peer Review Committee; PI, principal investigator;
SCA, Society of Cardiovascular Anesthesiologists; SIR, Society of Interventional Radiology; STS, Society of Thoracic Surgeons; SVM, Society for Vascular Medicine; SVS, Society for Vascular Surgery; and VA, Veterans Affairs.

Isselbacher et al
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JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. -, NO.

CLINICAL PRACTICE GUIDELINE

2022 ACC/AHA Guideline for the

ISSN 0735-1097/$36.00 https://doi.org/10.1016/j.jacc.2022.08.004

ABSTRACT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . - 1.2. Organization of the Writing Committee . . . . . . . . -

1.3. Document Review and Approval . . . . . . . . . . . . . . -

1.1. Methodology and Evidence Review . . . . . . . . . . . - 1.6. Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -

2. NORMAL ANATOMY, ABNORMAL ANATOMY, 6.5.5. Abdominal Aortic Aneurysms . . . . . . . . . . . -

2.1. Normal Aortic Anatomy . . . . . . . . . . . . . . . . . . . . . - 7. ACUTE AORTIC SYNDROMES . . . . . . . . . . . . . . . . . . -

2.2. Aortic Landing Zones . . . . . . . . . . . . . . . . . . . . . . . - 7.1. Presentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . -

3.1. Aortic Imaging Techniques to Determine 7.5. Management of IMH . . . . . . . . . . . . . . . . . . . . . . . -

Presence and Progression of Aortic Disease . . . . . -

3.2. Conventions of Measurements . . . . . . . . . . . . . . . - 7.6.1. PAU With IMH, Rupture, or Both . . . . . . . . -

3.2.1. Computed Tomography . . . . . . . . . . . . . . . . . - 7.6.2. Isolated PAU . . . . . . . . . . . . . . . . . . . . . . . . . -

3.2.4. Intravascular Ultrasound . . . . . . . . . . . . . . . . - 7.7. Traumatic Aortic Injury . . . . . . . . . . . . . . . . . . . . . -

3.2.5. Abdominal Ultrasound . . . . . . . . . . . . . . . . . . - 7.7.1. Initial Management of Blunt Traumatic

7.7.3. Long-Term Management and Surveillance

6.4.3. Surveillance for Medical Management . . . . -

9.2. Infectious Aortitis . . . . . . . . . . . . . . . . . . . . . . . . . . - capabilities, Multidisciplinary Aortic Team care is

Relationships With Industry and Other Entities 1. INTRODUCTION

TABLE 1 Associated Guidelines

Valvular heart disease ACC/AHA 20202

Large vessel vasculitis EULAR 20203

Blood cholesterol AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/ 20194

Congenital heart disease AHA/ACC 20195

Abdominal aortic aneurysm SVS 20186

High blood pressure ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/ 20187

Lower extremity peripheral artery disease AHA/ACC 20178

Descending thoracic aorta diseases ESVS 20179

Bicuspid aortic valves statement of clariﬁcation ACC/AHA 201610

Endovascular repair of traumatic thoracic aortic injury SVS 201112

Thoracic aortic disease ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM 201013

Coronary and other atherosclerotic vascular disease AHA/ACC 200614

Acute type A aortic dissection AATS 202115

Type B Aortic Dissection STS 202216

1.3. Document Review and Approval 1.4. Scope of the Guideline

Abbreviation Meaning/Phrase AVR aortic valve replacement

3D 3-dimensional BAAI blunt traumatic abdominal aortic injury

AAA abdominal aortic aneurysm BAV bicuspid aortic valve

AAS acute aortic syndrome BP blood pressure

ACEI angiotensin-converting enzyme inhibitor BSA body surface area

AHI aortic height index BTAI blunt traumatic aortic injury

AR aortic regurgitation BTTAI blunt traumatic thoracic aortic injury

ARB angiotensin receptor blocker CMR cardiac magnetic resonance

ASCA aberrant subclavian artery CoA coarctation of the aorta

ASCVD atherosclerotic cardiovascular disease CT computed tomography

ASI aortic size index CTA computed tomographic angiography

Continued on the next page Continued on the next page

MRA magnetic resonance angiography

F I G U R E 1 The Anatomy of the Aorta and Its Main Branches

“ectasia” is typically used to describe diffuse (rather than

2.3.1. Normalizing Aortic Root and Ascending Aortic Diameters

F I G U R E 5 Relative Risk of Aortic Dissection by Size Range

F I G U R E 6 Acute Aortic Syndromes

Classiﬁcation of Aortic Dissection Chronicity

F I G U R E 7 Classiﬁcation of Acute Aortic Dissection

branch vessels. The relationship of the true and false lu-

2.4.2. Intramural Hematoma

2.4.3. Penetrating Atherosclerotic Ulcer