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The Role of Echocardiography

in Hypertrophic Cardiomyopathy
Class I Recommendations Goals of Echocardiographic Assessment in Hypertrophic Cardiomyopathy (HCM)
for Echocardiography
in Hypertrophic Establish diagnosis & determine
Cardiomyopathy (HCM)* pattern of hypertrophy
Clinical diagnosis should be suspected with imaging evidence of
Suspected or a maximal end-diastolic wall thickness of >15 mm anywhere in the
established HCM left ventricle, absent another cause of hypertrophy in adults
Differentiate sigmoid septum (with ovoid cavity) versus reverse
1. TTE is recommended in initial curve (with crescent cavity) versus apical hypertrophic phenotypes
evaluation of all suspected HCM Massive left ventricular hypertrophy >30 mm in any left ventricular
2. In patients with established HCM
segment is a risk factor for sudden cardiac death (SCD) Sigmoid Reverse curve Apical
& no change in clinical status,
repeat TTE recommended every Evaluate global myocardial function
1 to 2 years
3. In patients with established Systolic dysfunction defined as LVEF <50%
HCM & change in clinical Strain abnormalities correlate with increased wall thickness
status, prompt repeat TTE is & delayed gadolinium enhancement by MRI
recommended
4. In patients who have undergone
septal reduction therapy, Establish presence & severity
TTE within 3-6 months of the of LVOT obstruction
procedure recommended to
evaluate procedural results Peak LVOT gradient of ≥50 mmHg at rest or with
provocation or exercise indicates obstruction
Differentiate SAM-mediated LVOT obstruction from
Screening & follow-up mid-ventricular obstruction (MVO; “dagger” shaped)
Peak MR velocity = 6.6 m/sec
Caution with contamination of LVOT signal with MR.
1. In first-degree relatives of MR velocity is higher & signal is of longer duration Peak LVSP = 4 ( 6.6 2 ) + LAP ( 10 mmHg )
patients with HCM, TTE is (spanning isovolumic contraction & relaxation) vs LVOT signal.
MR contour may be incomplete if Doppler signal not optimally aligned SBP = 113 mmHg
recommended as part of initial
family screening & periodic LVSP - SBP = LVOT gradient
Estimated LVOT gradient from MR signal calculated as:
follow-up LV Pressure - Systolic BP, where 174 + 10 = 184 - 113 = 71 mmHg
2. In individuals who are genotype- 6.6 m/sec
positive/ phenotype-negative, LV Pressure = 4 x ( Peak MR velocity ) 2 + LA Pressure
serial TTE is recommended at (assume 10 - 15 mmHg)
periodic intervals depending on
age & clinical status
Evaluate degree & direction of mitral
regurgitation, & intrinsic structure of
Provocative testing mitral valve & papillary muscles
1. In patients with HCM & resting MR caused by LVOT obstruction results from SAM & results
LVOT gradient <50 mmHg, TTE in a jet direction that is posterior or lateral in orientation &
with provocative maneuvers predominantly mid-to-late systolic
recommended
Central or anterior jets should prompt further evaluation for
2. In symptomatic patients with HCM intrinsic valvular or papillary abnormalities
without resting or provocable
LVOT gradient ≥50 mmHg on
TTE, exercise TTE recommended Evaluate for LV apical aneurysm
for detection & quantification of
dynamic LVOT obstruction TTE with ultrasound enhancing agent should be performed in
patients with HCM with suspected apical or mid-ventricular
hypertrophy to evaluate for apical aneurysms
Septal reduction therapies Presence of an LV apical aneurysm is an established
clinical risk factor for SCD
1. For patients with HCM
undergoing surgical septal
myectomy, intraoperative TEE
recommended to assess mitral
Plan & guide septal reduction therapies & subsequently assess their efficacy
valve anatomy & function & LA
adequacy of septal myectomy Preoperative Identify appropriate septal
measurements include: perforator that supplies
2. For patients with HCM A) IVS maximum thickness B SAM-septal contact
undergoing alcohol septal IVS (by TTE or TEE)
ablation, TTE or intraoperative B) Anterior leaflet length Inappropriate targets:
LV
TEE with intracoronary C) Apical extent of septal bulge A • Distal Septum
ultrasound-enhancing contrast
injection of candidate’s septal D) Distance from aortic annulus • LV Papillary Muscles
perforator(s) is recommended to mitral-septal contact Pre-myectomy measurements • RV Papillary Muscles Alcohol septal ablation

Abbreviations HCM Hypertrophic cardiomyopathy LVOTO Left ventricular outflow tract obstruction MR Mitral regurgitation SAM Systolic anterior motion
LVOT Left ventricular outflow tract LVSP Left ventricular systolic pressure MVO Mid-ventricular obstruction SCD Sudden cardiac death

* ACC/AHA Guideline Document (See full citation on back)

Nagueh SF, Bierig SM, Budoff MJ, Desai M, Dilsizian V, Eidem B, Goldstein SA, Hung J, Maron MS, Ommen SR, Woo A; American Society of Echocardiography; American Society of Nuclear Cardiology;
Society for Cardiovascular Magnetic Resonance; Society of Cardiovascular Computed Tomography. American Society of Echocardiography clinical recommendations for multimodality cardiovascular
imaging of patients with hypertrophic cardiomyopathy: Endorsed by the American Society of Nuclear Cardiology, Society for Cardiovascular Magnetic Resonance, and Society of Cardiovascular
Computed Tomography. J Am Soc Echocardiogr. 2011 May;24(5):473-98.
Nicoara A, Skubas N, Ad N, Finley A, Hahn RT, Mahmood F, Mankad S, Nyman CB, Pagani F, Porter TR, Rehfeldt K, Stone S, Taylor B, Vegas A, Zimmerman KG, Zogbhi WA, Swaminathan M. Guidelines for
the use of transesophageal echocardiography to assist with surgical decision-making in the operating room: a surgery-based approach from the American Society of Echocardiography in collaboration
with Society of Cardiovascular Anesthesiologists and the Society of Thoracic Surgeons. J Am Soc Echocardiogr. 2020;33:692-734.
Silbiger JJ. Abnormalities of the Mitral Apparatus in Hypertrophic Cardiomyopathy: Echocardiographic, Pathophysiologic, and Surgical Insights. J Am Soc Echocardiogr. 2016 Jul;29(7):622-39.

Poster Authors: Karan Kapoor, MD; Allison G Hays, MD, FASE. Design and illustration by medmovie.com
© Copyright 2021 The American Society of Echocardiography
 Recommended Evaluation & Testing
for Hypertrophic Cardiomyopathy
Family History of Hypertrophic Cardiomyopathy (HCM) or Suspected HCM

Perform Diagnostic Evaluation (ECG, Imaging)

Phenotype Negative Phenotype Positive

Risk factors for SCD Perform Comprehensive Baseline Evaluation

with SCD risk assessment (consider ESC guidelines)
• Unexplained Syncope
Family with a known • Massive LVH Stress testing
• Family history SCD • to determine baseline functional status
pathogenic or likely • Apical aneurysm • to clarify symptom status
• EF <50%
pathogenic variant? • Extensive LGE on CMR
• to confirm presence and quantify severity of LVOTO

Class I Class IIb

Yes No
Every 1-2 years or sooner
if change in symptoms Every 3-5 years
Perform serial evaluation of clinical Perform CMR for
status & SCD risk with SCD risk assessment or to
• Clinical assessment evaluate for any suspected
Patient has
No • Echo morphologic changes
family variant? • Holter

Yes, or unknown If Symptomatic If Asymptomatic

Class I Class III: No Benefit Treadmill or Bike Exercise Testing

Every 2-3 years
Special considerations:
Treadmill exercise
Screening ECG & Echo Further clinical or • Stress echo if gradient <50 mmHg or CPET testing for
• CPET if considering advanced HF assessment of functional
at the intervals in the genetic testing is not
therapies status
table below recommended

Screening Asymptomatic First-Degree Relatives of Patients with HCM Class (Strength) of

Age of First-Degree Relative Initiation of Screening Surveillance Interval
Recommendation
Children & adolescents from genotype-positive At the time of diagnosis in Every 1-2 years Class I (STRONG) Benefit >>> Risk
family and/or family with early onset HCM another family member
All other children & adolescents At any time after the diagnosis in Every 2-3 years Class IIb (WEAK) Benefit ≥ Risk
the family (no later than puberty)
Adults At the time of diagnosis in Every 3-5 years
another family member
Class III: No Benefit Benefit = Risk

Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron
MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With
Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on
Clinical Practice Guidelines. J Am Coll Cardiol. 2020 Dec 22;76(25):3022-3055.