Valve-Sparing Aortic Root Replacement in Loeys-Dietz Syndrome

Valve-Sparing Aortic Root Replacement in Loeys-Dietz Syndrome ADULT CARDIAC Nishant D. Patel, MD, George J. Arnaoutakis, MD, Timothy J. George, MD, Jeremiah G. Allen, MD, Diane E. Alejo, BA, Harry C. Dietz, MD, Duke E. Cameron, MD, and Luca A. Vricella, MD Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland Background. Loeys-Dietz syndrome (LDS) is a recently Thirty patients (97%) underwent reimplantation proce- recognized aggressive aortic disorder characterized by dures using a Valsalva graft. There were no operative root aneurysm, arterial tortuosity, hypertelorism, and deaths. Mean follow-up was 3.6 years (range, 0 to 7 bifid uvula or cleft palate. The results of prophylactic years). One patient required late repair of a pseudoaneu- root replacement using valve-sparing procedures (valve- rysm at the distal aortic anastomosis, and 1 had a conver- sparing root replacement [VSRR]) in patients with LDS sion to a David reimplantation procedure after a Florida is not known. sleeve operation. No patient suffered thromboembolism Methods. We reviewed all patients with clinical and or endocarditis, and 1 (3%) patient experienced greater genetic (transforming growth factor-␤ receptor muta- than 2ⴙ late aortic insufficiency. No patient required late tions) evidence of LDS who underwent VSRR at our aortic valve repair or replacement. institution. Echocardiographic and clinical data were Conclusions. Loeys-Dietz syndrome is an aggressive obtained from hospital and follow-up clinic records. aortic aneurysm syndrome that can be addressed by Results. From 2002 to 2009, 31 patients with a firm prophylactic aortic root replacement with low operative diagnosis of LDS underwent VSRR for aortic root aneu- risk. Valve-sparing procedures have encouraging early rysm. Mean age was 15 years, and 24 (77%) were children. and midterm results, similar to those in Marfan syn- One (3%) patient had a bicuspid aortic valve. Preopera- drome, and are an attractive option for young patients. tive sinus diameter was 3.9 ⴞ 0.8 cm (z score 7.0 ⴞ 2.9) (Ann Thorac Surg 2011;92:556 – 61) and 2 (6%) had greater than 2ⴙ aortic insufficiency. © 2011 by The Society of Thoracic Surgeons A ortic root aneurysms are rare in children; they are usually associated with connective tissue disorders and conotruncal congenital heart disease and may com- in the receptors for transforming growth factor (TGF)-␤ [7]. Loeys-Dietz syndrome (LDS) is characterized by aggressive vascular disease, arterial tortuosity and aneu- plicate some cardiovascular surgical operations such as rysm, hypertelorism, and bifid uvula or cleft palate. the Ross procedure, Norwood procedure, and arterial Patients with LDS experience aortic dissection and rup- switch. Aortic root replacement may be indicated in some ture at smaller aortic diameters and at a younger age than children to prevent aneurysm rupture, aortic dissection, do patients with Marfan syndrome. We have embarked and valvar incompetence. on a program of early prophylactic aortic root replace- Traditionally the Bentall procedure [1] has been the ment to prevent vascular catastrophe in LDS. This review operation of choice for aortic root disease and has dem- was conducted to evaluate the safety and early results of onstrated excellent long-term outcomes [2– 4]. However this surgical program. an aortic valve with a mechanical prosthesis necessitates long-term anticoagulation and bioprosthetic valves have limited durability in young patients. These consider- Material and Methods ations make valve-sparing aortic root replacement Study Design, Patient Selection, and Patient Variables (VSRR), originally described by Sarsan and Yacoub [5] After institutional review board approval, data were and David and Feindel [6], an attractive option for chil- collected retrospectively for all patients with LDS who dren with aortic root aneurysms. underwent VSRR between 2002 and 2009 at the Johns Recently Loeys and Dietz described an autosomal- Hopkins Hospital. Data were obtained from medical and dominant inherited syndrome resulting from mutations electronic patient records. A waiver for individual con- Accepted for publication April 1, 2011. sent was granted by our institutional review board. Presented at the Fifty-seventh Annual Meeting of the Southern Thoracic All patients who underwent VSRR had transthoracic Surgical Association, Orlando, FL, Nov 3– 6, 2010. echocardiograms to assess preoperative and postopera- Address correspondence to Dr Vricella, Johns Hopkins Medical Institutions, tive aortic valve function. Intraoperative transesophageal 600 N Wolfe St, Blalock 618, Baltimore, MD 21287; e-mail: lvricella@jhmi. echocardiograms were routinely obtained. Echocardio- edu. graphic data included maximum aortic sinus diameter, © 2011 by The Society of Thoracic Surgeons 0003-4975/$36.00 Published by Elsevier Inc doi:10.1016/j.athoracsur.2011.04.003 Ann Thorac Surg PATEL ET AL 557 2011;92:556 – 61 VSRR IN LOEYS-DIETZ SYNDROME sinotubular junction diameter, and annular diameter lar sutures are passed inside-out through the bottom of with respective z scores (number of SDs away from mean the Valsalva graft 120 degrees apart from each other. The for aortic dimensions based on patient body surface stay sutures are drawn up within the graft, which is area), as well as the degree of aortic insufficiency (0 to lowered around the valve complex, and the 3 subannular 4⫹). Clinical follow-up data, including postoperative aor- sutures are tied. ADULT CARDIAC tic insufficiency and need for reoperation on the aortic The commissures are fixed to the sinotubular junction root, were obtained by telephone interviews with the of the graft using 3 4-0 polypropylene pledgetted mat- patient, family members, and primary care physicians. tress sutures. In most patients the commissure height is Some patients with LDS and aortic root aneurysm/ level with the sinotubular ridge of the graft. In patients dissection were not offered VSRR and instead had root with very large roots and tall commissures, fixation can replacement with composite grafts. Our relative contra- be just above the ridge. The valve is now properly indications for VSRR include marked leaflet fenestration positioned within the graft by the “3 below” and the “3 and asymmetry, acute aortic dissection in unstable pa- above” fixation sutures. Starting at the bottom of each tients, and a giant root with marked leaflet irregularities. sinus and sewing upward to the commissure tops, con- For these patients, a Bentall procedure was performed. In tinuous 4-0 polypropylene sutures are used to fix the addition, we do not routinely perform VSRR on bicuspid sinus remnant and annulus within the graft. This is the aortic valves with extensive calcification, severe prolapse, hemostatic suture line. marked fenestrations, or a combination of these Holes are cut in the graft opposite the coronary artery anomalies. buttons. We encircle the coronary buttons with polytet- The diagnosis of LDS was confirmed both by genetic rafluoroethylene (Teflon) felt “lifesaver” pledgets. The analysis and clinical phenotype in collaboration with left and right coronary anastomoses are completed with colleagues in the McKusick Department of Medical Ge- 4-0 polypropylene. Finally the distal graft is anastomosed netics and the Connective Tissue Disorders Clinic at the to the aorta using continuous 4-0 polypropylene suture Johns Hopkins Hospital. and an external felt strip. Air is evacuated from the heart, the aortic cross-clamp is removed, and the heart is Operative Technique resuscitated. Patients underwent a modified David V procedure in which the native aortic valve was resuspended within a Statistical Analysis Dacron tube graft with prefashioned pseudosinuses Statistical analyses were conducted with Stata version 9.0 (Gelweave Valsalva graft; Vaskutek, Renfrewshire, Scot- software package (Stata Corporation, College Station, land, UK). One patient had a reimplantation procedure TX). Survival and freedom from reoperation of the aortic using a straight Dacron graft because of unavailability of root were estimated using the Kaplan-Meier method. All an appropriate small-sized Valsalva graft. Our operative continuous variables are presented as mean ⫾ SD, unless technique has been described in detail elsewhere [8]. All otherwise noted. operations are performed through a median sternotomy. Patients are placed on cardiopulmonary bypass using aortic and bicaval venous cannulation. A vent is placed in Results the left atrium through the right superior pulmonary Preoperative Clinical Characteristics vein. After aortic clamping and cardioplegic arrest, the A total of 31 patients with a firm diagnosis of LDS aorta is divided above the sinotubular ridge. Using com- underwent VSRR between 2002 and 2009. Preoperative mercial valve sizers, we determine the diameter of the patient characteristics are shown in Table 1. Mean age at sinotubular junction that provides optimal leaflet appo- operation was 14.6 ⫾ 12.8 years (median, 12.7 years; sition and then select a Valsalva graft that is 2 to 3 mm range, 9 months to 60 years), and 14 (45.2%) were female larger because the prosthesis fits outside the aortic valve complex. The base of the aorta is dissected externally down to a subannular level. This is followed by excision Table 1. Baseline Clinical Characteristics of sinus tissue, leaving an approximately 4-mm to 5-mm Variable N ⫽ 31 (%) sinus remnant attached to the annulus. The coronary Mean age at operation (years) 14.6 ⫾ 12.8 arteries are mobilized widely. Three 3-0 braided polyes- ter pledgetted horizontal mattress sutures are passed Female patient 14 (45.2) under the annulus at the nadir of each aortic leaflet from Pediatric (⬍18 years) 24 (77.4) inside outward. Our procedure differs from the original Previous cardiac surgery 5 (16.1) David I reimplantation technique in that only 3 subannu- Bicuspid aortic valve 1 (3.2) lar sutures are placed. The subannular sutures anchor Preoperative maximum sinus 3.9 ⫾ 0.8 (z ⫽ 7.0 ⫾ 2.9) the graft and ensure that the entire aortic valve complex diameter (cm) is within the sinus segment of the Valsalva graft. The Preoperative sinotubular junction 2.8 ⫾ 0.8 (z ⫽ 4.6 ⫾ 2.5) diameter (cm) proximal collar of the graft is trimmed to 2 rings. The Preoperative annular diameter (cm) 2.2 ⫾ 0.5 (z ⫽ 3.8 ⫾ 2.3) distal end of the graft is usually trimmed to 4 to 5 rings Preoperative ⬎ 2⫹ aortic 2 (6.5) but may be left longer if more extensive replacement of insufficiency the ascending aorta or arch is necessary. The 3 subannu-  558 PATEL ET AL Ann Thorac Surg VSRR IN LOEYS-DIETZ SYNDROME 2011;92:556 – 61 patients. Twenty-four (77.4%) were younger than 18 Table 3. Preoperative and Postoperative Sinus and Annular years of age at the time of operation. One (3.2%) had a Diameters bicuspid aortic valve. Five (16.1%) patients had had Variable Preoperative Postoperative p Value previous cardiac surgery. All were New York Heart Association class I preoperatively. Annulus (cm) 2.2 ⫾ 0.5 2.2 ⫾ 0.4 1.0 ADULT CARDIAC Preoperative sinus diameter was 3.9 ⫾ 0.8 cm (z score Annulus z score 3.8 ⫾ 2.3 2.6 ⫾ 3.1 0.08 7.0 ⫾ 2.9), sinotubular junction diameter was 2.8 ⫾ 0.8 Maximum sinus (cm) 3.9 ⫾ 0.8 3.0 ⫾ 0.4 ⬍ 0.001 cm (z score 4.6 ⫾ 2.5), and annular diameter was 2.2 ⫾ Maximum sinus z score 7.0 ⫾ 2.9 2.6 ⫾ 2.6 ⬍ 0.001 0.5 cm (z score 3.8 ⫾ 2.3). Two (6.5%) patients had greater than 2⫹ aortic insufficiency on preoperative echocardiography. ameters with respective z scores are shown Table 3. A 1-year-old patient who initially underwent a Florida Operative Data sleeve procedure experienced a coronary artery button Operative data are displayed in Table 2. One patient aneurysm and then had a VSRR with the Valsalva graft 4 (3.2%) underwent David I reimplantation with a straight years later. One patient experienced greater than 2⫹ tube Dacron graft, and 30 (96.8%) patients had reimplan- aortic insufficiency 4.3 years after reimplantation with the tation procedures using a Valsalva graft. Mean cardio- Valsalva graft. Freedom from late aortic valve repair or pulmonary bypass and cross-clamp times were 144.4 ⫾ replacement was 100% at 1, 3, and 5 years after 21.7 and 101.3 ⫾ 17.5 minutes, respectively. The most reimplantation. common prosthesis size was 24 mm and was used in 13 No patient experienced thromboembolism or endocar- (41.9%) patients. ditis at follow-up and no patient had coronary artery Fifteen (48.4%) patients had patent foramen ovale anastomotic dehiscence. closure at the time of VSRR. Three (9.7%) had concomi- tant patent ductus arteriosus ligation, 3 (9.7%) had atrial septal defect closure, and 1 (3.2%) had a ventricular Comment septal defect closure. Two (6.5%) patients had concomi- Aortic root replacement with a mechanical valve has tant aortic valve repair at the time of VSRR. been the gold standard of care for patients with aortic root aneurysms for more than 40 years. It has been a safe, Survival and Functional Class reproducible, and durable operation, but nonetheless There were no operative or late deaths. Kaplan-Meier results in a low, constant risk of thromboembolism, survival was 100% at 7 years. All patients were New York endocarditis, and anticoagulant-related hemorrhage [2– Hospital Association class I at follow-up. 4]. Implications for lifestyle modification are not insignif- icant for many patients. For these reasons, we and others In-Hospital Complications have adopted and selectively applied alternative valve- Four (12.9%) patients experienced pneumothorax post- preservation surgical strategies for aortic root replace- operatively. Postoperative infection rates were low. Two ment over the last 15 years. (6.5%) patients had postoperative pneumonia. There Low operative mortality and good midterm results were no postoperative urinary tract infections or sternal have been reported after VSRR in patients with aortic wound infections. aneurysm [9 –18]. In our initial experience, we used the remodeling technique with scalloped “Dacron tongues” Late Outcomes because of the theoretic advantage of preserved sinuses Mean follow-up was 3.7 years (range, 0 to 7 years). One [19 –23]. However we observed a trend toward late annu- 8-year-old female patient who underwent reimplantation lar dilatation and aortic insufficiency likely caused by with a 24-mm Valsalva graft required repair of a distal splaying of the Dacron tongues and loss of leaflet appo- pseudoaneurysm 29 months postoperatively. Preopera- sition height. This has been observed particularly in tive and postoperative maximum sinus and annular di- patients with connective tissue disorders. Our protocol changed to the reimplantation procedure in 2002 when the Gelweave Valsalva graft, a Dacron graft with prefash- Table 2. Operative Data ioned pseudosinuses, became commercially available. We believe that the reimplantation technique using the Variable N ⫽ 31 (%) Valsalva graft preserves sinuses, stabilizes the aortic root Mean cross-clamp time (min) 101.3 ⫾ 17.5 below the nadir of the sinuses, and improves hemostasis. Mean cardiopulmonary bypass time (min) 144.4 ⫾ 21.7 Results of VSRR in Marfan syndrome have been re- Concomitant procedures ported, but such results are lacking for LDS [24]. LDS Aortic valve repair 2 (6.5) results from mutations in the gene coding for receptors Patent foramen ovale closure 15 (48.4) for TGF-␤, which lead to abnormal TGF-␤ signaling and Atrial septal defect 3 (9.7) abnormal collagen deposition in the aortic wall. The Ventricular septal defect 1 (3.2) latter, along with elastin disarray, results in a structurally weak medium that predisposes to aortic aneurysm, dis- Patent ductus arteriosus ligation 3 (9.7) section, and rupture [24]. Ann Thorac Surg PATEL ET AL 559 2011;92:556 – 61 VSRR IN LOEYS-DIETZ SYNDROME Patients with LDS present with a characteristic pheno- after reimplantation with the Valsalva graft. At the time type of hypertelorism, bifid uvula or cleft palate (or both), of the original operation we noted marked leaflet fenes- and arterial tortuosity [24]. Like their Marfan counter- trations, but these were not considered severe enough to parts, patients with LDS share a predisposition for aneu- warrant valve replacement. Although this patient will rysm of the aortic root and risk of rupture and dissection, likely require valve replacement in the years ahead, the ADULT CARDIAC even in childhood. At our institution patients with LDS remainder of the postoperative cohort has enjoyed excel- are referred for surgical intervention at a younger age lent freedom from significant valve regurgitation or and smaller aortic root diameters than patients with reoperation. Marfan syndrome in concordance with the more aggres- Surgeon experience and patient selection are critical sive vascular phenotype of the former. for good outcomes. Our operative technique for reim- We reported our initial surgical experience in patients plantation using the Valsalva graft is simple and repro- with LDS in 2004 [25]. In that study 14 of the 71 patients ducible. We recommend a Bentall procedure for patients with LDS underwent VSRR at 2 separate institutions with severe aortic insufficiency who have marked leaflet (Johns Hopkins and University of Ghent). One pediatric asymmetry or leaflet fenestrations as well as for patients patient initially underwent VSRR/arch replacement at with bicuspid valves and significant stenosis, thickening, Ghent Hospital, required subsequent Bentall operation, prolapse, or fenestrations. Composite grafts are likely to and died 11 years after the initial VSRR procedure. This play a role in the management of patients with LDS; over initial experience was instructive in the importance of the time course of this study, 30 patients with LDS had an early prophylactic root replacement and frequent sur- aortic root replacement with a composite graft. veillance of the vascular tree. Of the 71 patients, fatal For postoperative surveillance, we recommend that all intracerebral and aortic catastrophies occurred in 3 pa- tients younger than 10 years of age. Furthermore, aortic patients who receive VSRR undergo echocardiography, rupture occurred at diameters less than 4.5 cm, which is computed tomography (CT), or magnetic resonance im- a rare event in Marfan syndrome. aging 6 months postoperatively and annually thereafter For most adult patients with ascending aortic aneu- to monitor aortic root diameter and valve competence. rysm, the threshold for elective aortic root replacement is Because of the aggressive nature of LDS and the risk of an aortic diameter of greater than 5.0 cm, rate of aortic rupture and dissection at smaller aortic root diameters, root growth greater than 1.0 cm/year, or worsening aortic we recommend echocardiography every 3 to 6 months for insufficiency. Indications for VSRR for patients with LDS 1 year after surgery and every 6 months thereafter. Given are not as well defined. Given the much greater risk of the widespread involvement of the arterial tree in pa- aortic catastrophe in LDS, our current threshold for aortic tients with LDS, we also recommend annual head-to- root replacement in adults is an aortic root diameter pelvis body CT scans. greater than 4.0 cm or root expansion greater than 0.5 Valve-sparing root replacement is a safe and effective cm/year. For children with severe craniofacial abnormal- surgical option for the management of aortic root aneu- ities (Loeys-Dietz type I) we advise surgery at an aortic rysm in patients with LDS. Because of the malignant and root z score greater than 3.0 or an aortic root expansion aggressive nature of this aortopathy, early recognition of greater than 0.5 cm/year. For children with mild cranio- the syndrome by clinical phenotype or genetic analysis is facial abnormalities (Loeys-Dietz type II), we recommend critical. We recommend the reimplantation technique surgery at an aortic root z score greater than 4.0 or an with the Valsalva graft for prophylactic root replacement aortic root that expands greater than 0.5 cm/year. because of improved stabilization of the annulus, better We are also reconsidering our management of the hemostasis, and preservation of sinuses. Although long- distal ascending aorta and proximal arch at the time of term results are not yet available, midterm outcomes are root replacement. None of the patients in this series had encouraging. an arch or hemiarch replacement because the arch diam- eters were normal at the time of the valve-sparing pro- cedure. However several patients experienced dilatated References distal ascending aortas and proximal arches within a few 1. Bentall HH, De Bono A. A technique for complete replace- years postoperatively; this too is an unusual event in ment of the ascending aorta. Thorax 1968;23:338 –9. Marfan syndrome. Further experience may suggest that 2. Gott VL, Greene PS, Alejo DE, et al. Replacement of the more aggressive proximal aortic resection is indicated in aortic root in patients with Marfan’s syndrome. N Engl some patients with LDS. J Med 1999;340:1307–13. 3. Gott VL, Cameron DE, Alejo DE, et al. Aortic root replace- In this present study all but 1 patient had a reimplan- ment in 271 Marfan patients: a 24-year experience. Ann tation procedure using the Valsalva graft. A straight Thorac Surg 2002;73:438 – 43. Dacron graft was used in that 1 patient because the aortic 4. Cameron DE, Alejo DE, Patel ND, et al. Aortic root replace- root diameter was smaller than 24 mm, the smallest ment in 372 Marfan patients: evolution of operative repair commercially available Valsalva graft. over 30 years. Ann Thorac Surg 2009;87: 1344 –50. 5. 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Ann Thorac Surg 2007;83:S757– valve-sparing procedures. Ann Thorac Surg 2008;85:2003–11. 63. DISCUSSION DR THORALF SUNDT (Rochester, MN): I hate to shake every- valve-sparing operation and 30 had a composite root replace- body up twice in the same day between the picture of me from ment, so roughly 50:50. earlier this morning in Keith’s address and now my appearance in a congenital session, but I appreciate the invitation from the DR SUNDT: The next question relates to the uniqueness of this program committee nonetheless. I have a long-standing interest disease, a disease that your group is most familiar with but in surgery for congenital heart disease, and the valve-sparing others are probably less so. It really gets to the question “Why root procedure is one of my favorite operations as well. write this paper at all?” The implication of performing the So congratulations Dr Patel. It was a beautiful presentation. In analysis is that in some way these patients are going to be addition, I should point out that the Tiki Award as originally different from Marfan patients, for example, so share with us conceived can be given for outstanding slides as well, and this is more of your thinking today about this disease. Are there some one of the most clear, beautiful presentations I have seen in a special concerns you think about when you are doing a valve- long time. So I would recommend that the Tiki Committee sparing root procedure in Loeys-Dietz syndrome? Given the consider giving the award to somebody who did it right and not uniqueness of the tissues and such, do you modify the opera- always for somebody who did it wrong. tion? For example, are you more aggressive with the distal Congratulations to you, congratulations to Duke, to Luca, and anastomosis? Do you do a hemiarch replacement or even a total to Bill for mentoring you and bringing you along. It is critical to arch replacement with the idea that these patients are subject to the health of our specialty that residents in general surgery be subsequent aneurysmal dilatation or dissection of the arch? brought along to become interested in this specialty. What is your attitude about the leaflets? My experience with some patients with Marfan syndrome is that there is a spectrum My first questions for you are, “How many patients during of how “stretchy” the leaflets are. Is this a concern in Loeys- this period of time with Loeys-Dietz did not have a valve- Dietz syndrome? What keeps your group up at night worried sparing root operation? What percentage, roughly, of patients about late outcomes. with Loeys-Dietz are going to turn out to be candidates for Congratulations on a really beautiful presentation. valve-sparing operations?” Duke has mentioned before that he has reasonably strict criteria about the amount of leaflet fenes- tration that he will allow in a patient in whom he will do a DR PATEL: Thank you again for those very important questions. valve-sparing root. What percentage of Loeys-Dietz are candi- With regard to why we did this study and what we were anticipating with the results for this study, we do believe that dates do you think? these patients have very, very malignant tissues. Our initial experience back in 2004, which we reported in the Annals of DR PATEL: Thank you very much for your comments. Over the Thoracic Surgery, was 71 patients we described who had Loeys- eight-year time period, from 2002 to 2009, 31 patients had a Dietz syndrome; roughly half of them were from the University Ann Thorac Surg PATEL ET AL 561 2011;92:556 – 61 VSRR IN LOEYS-DIETZ SYNDROME of Ghent, where Dr Loeys is located, and the other half were DR ROBERT STEWART (Cleveland, OH): I echo Dr Sundt’s from our institution with Hal Dietz. Of those 71 patients, 5 compliments on your wonderful presentation. My question children less than 10 years of age with roots less than 4 cm died relates to a patient with Loeys-Dietz syndrome who requires a of a fatal aortic catastrophe, so given that very early data, we cardiac operation for a lesion other than in their aorta. If such a decided to be very aggressive with proceeding with root replace- patient has aortic dilatation, but it does not quite meet your ADULT CARDIAC ment and monitoring them postoperatively. criteria for repair, do you feel that continued dilatation is Typically for Marfan syndrome we will obtain echocardio- inevitable and if so, would you consider replacing the aortic root grams at 6 months and then annually thereafter. These patients when you are fixing the other lesion? are followed every 3 months with echocardiography and then every year with CT imaging from the head all the way down to DR PATEL: Well, that is interesting in this set of patients the pelvis. because as you saw in my clinical characteristics slide there, With regard to replacing the arch, none of the patients in this there were 2 patients who were fairly elderly when they received series have had abnormal arch diameters and none of them have their operation; 1 was 60 years of age. We do believe that these had prophylactic arch replacement, but we have seen some of patients will eventually have dilatated aortic roots. At present, I our composites come back into the clinic with some arch and would say, from what Dr Cameron and Dr Vricella have told me, descending thoracic aneurysms. At this time we are hesitant to that we should proceed with replacing their roots given these replace the arch and proceed with a more aggressive descending very early criteria. These criteria that we have developed aren’t thoracic resection because we will be subjecting these patients to based on any data, unfortunately, because we really don’t have a much more difficult operation as well as subjecting their very that data available to us yet. A lot of the natural history of this friable tissues to more suture lines. What we are hoping is that disease is still unknown. Hopefully with more long-term fol- with more long-term data we will be able to identify specific low-up with these patients, as well as patients who have not patients who may benefit from a more aggressive surgical undergone surgery, we will be able to better answer that intervention. question.