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Interactive CardioVascular and Thoracic Surgery 12 (2011) 415–419

� 2011 Published by European Association for Cardio-Thoracic Surgery

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Institutional report - Congenital

Valve-sparing aortic root replacement in children:intermediate-term results�

Nishant D. Patel, George J. Arnaoutakis, Timothy J. George, Jeremiah G. Allen, Diane E. Alejo,Harry C. Dietz, Duke E. Cameron, Luca A. Vricella*

Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, MD 21287, USA

Received 28 September 2010; received in revised form 6 November 2010; accepted 8 November 2010

Abstract

Valve-sparing root replacement (VSRR) is an attractive option for aortic aneurysm in children with low-operative risk, but mid- and late-term results are not yet known. Between 1997 and 2009, 56 children (mean age 11.5 years) underwent VSRR at our institution. Twenty-six(46.4%) had Marfan syndrome and 24 (42.8%) had Loeys–Dietz syndrome. Mean preoperative max sinus diameter was 4.2"0.8 cm (Z-score7.7"2.9). Five (8.9%) had )2q aortic insufficiency (AI). Two (3.6%) underwent David I reimplantation with a straight-tube, 12 (21.4%)had a Yacoub remodeling procedure, and 42 (75.0%) had reimplantation using a Valsalva-graft. There were one (1.8%) operative and three(5.4%) late deaths. One patient required reoperation for bleeding and one required late repair of a distal pseudoaneurysm. Mean follow-up was 5.2 years (range 0–12 years). No patients suffered thromboembolic events or had endocarditis. Of the 12 remodeling patients, four(33.3%) developed )2q AI and required aortic valve repair or replacement. No patient developed )2q AI after reimplantation. VSRR inchildren is a safe alternative to aortic root replacement with mechanical or biological prostheses. In this particular group of patients withconnective tissue disorders and proclivity toward annular dilation and late AI, reimplantation is superior to remodeling.� 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

Keywords: Aortic aneurysm; Pediatric; Aortic root replacement

1. Introduction

Aortic root aneurysms in children often result from con-nective tissue disorders, such as Marfan syndrome andLoeys–Dietz syndrome, or after operative management ofcongenital heart disorders. Aortic root replacement is indi-cated in these children to avoid the risk of progressivevalvular incompetence, aneurysm rupture, and dissection.

Composite aortic root replacement with a mechanicalprosthesis w1x is the gold standard for patients with aorticroot aneurysms w2–4x, but carries the inherent risks of life-long anticoagulation. Over the past two decades, valve-sparing aortic root replacement (VSRR) techniques w5, 6xhave been utilized by many centers as an alternative tocomposite replacement w7–11x. While long-term data inadults have shown good results w7–11x, outcomes followingVSRR for children with aortic root aneurysm are lacking.We present our 14-year single institution experience withVSRR in pediatric patients.

� Presented at the 24th Annual Meeting of the European Association forCardio-Thoracic Surgery, Geneva, Switzerland, September 11–15, 2010.

*Corresponding author. Pediatric Cardiac Surgery, Division of Cardiac Sur-gery, Johns Hopkins Medical Institutions, 600 North Wolfe Street, Blalock618, Baltimore, MD 21287, USA. Tel.: q1-443-2871262.

E-mail address: lvricella@jhmi.edu (L.A. Vricella).

2. Methods

2.1. Study design, patient selection, and patientvariables

Following Institutional Review Board approval, data werecollected retrospectively for all children -18 years of agewho underwent VSRR between 1997 and 2009 at The JohnsHopkins Hospital. Data were obtained from medical elec-tronic patient records. A waiver for individual consent wasgranted by our Institutional Review Board.

All VSRR patients had transthoracic echocardiograms toassess preoperative and postoperative aortic valve func-tion. Intraoperative transesophageal echocardiograms wereroutinely obtained. Echocardiographical data included aor-tic root diameters with respective Z-scores, as well as thedegree of aortic insufficiency (AI) (0–4q). Clinical follow-up data were obtained by telephone interview with thepatient, family members, andyor primary care physicians.

2.2. Operative technique

Our operative technique for VSRR has been described indetail elsewhere w12x. The first 12 children in our VSRRexperience had a remodeling procedure (Yacoub); eight ofthese children underwent a David III remodeling procedurewhere the suture line along the Nadir of the non-coronarysinus was reinforced with a Teflon-felt strip (Impra, Subsi-diary of L.R. Bard, Tempe, AZ, USA). Two children had

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Table 1. Baseline clinical characteristics

ns56 (%)

Mean age at operation (years) 11.5"5.9Male 34 (60.7)Marfan syndrome 26 (46.4)Loeys–Dietz syndrome 24 (42.8)No connective tissue disorder 3 (5.4)Non-specific connective tissue disorder 2 (3.6)Previous cardiac surgery 5 (8.9)Preoperative max sinus diameter (cm) 4.2"0.8 (Zs7.7"2.9)Preoperative STJ diameter (cm) 3.1"0.9 (Zs5.6"3.2)Preoperative annular diameter (cm) 2.5"0.4 (Zs3.5"2.3)Preoperative )2q aortic insufficiency 5 (8.9)

STJ, sinotubular junction Fig. 1. Kaplan–Meier survival.

reimplantation within a straight-tube Dacron graft (DavidI). The remainder underwent a modified David V procedurewhere the native aortic valve was resuspended within aDacron-tube graft with prefashioned pseudosinuses (Gel-weave Valsalva graft). Since 2002, we have preferred thereimplantation technique using the Valsalva graft.

2.3. Statistical analysis

Statistical analyses were conducted with Stata Version 9.0(Stata Corp, College Station, TX, USA) software package.Survival and freedom from reoperation of the aortic rootwas estimated using the Kaplan–Meier method, and com-parisons between groups made with the log rank test. Allcontinuous variables are presented as mean"standarddeviation (S.D.), unless otherwise noted.

3. Results

3.1. Preoperative clinical characteristics

Fifty-six children underwent VSRR between 1997 and 2009.Preoperative data are shown in Table 1. Mean age at ope-ration was 11.5"4.9 years (range nine months – 17.5years), and 34 (60.7%) were male. Twenty-six (46.4%)children had Marfan syndrome, 24 (42.8%) had Loeys–Dietzsyndrome, two (3.6%) had non-specific connective tissuedisorders, three (5.4%) had no connective tissue disorders,and one (1.8%) had Ehlers–Danlos syndrome. Four (7.1%)had a bicuspid aortic valve (two Marfan patients, oneLoeys–Dietz patient, and one patient without connectivetissue disorder). Five (8.9%) patients had previous cardiacsurgery: one (1.8%) patient without connective tissue dis-ease had VSRR after successful arterial switch, one (1.8%)patient with a non-specific connective tissue disorder hadVSRR after successful repair for tetralogy of Fallot, one(1.8%) Loeys–Dietz patient had VSRR following a FloridaSleeve procedure, one (1.8%) Loeys–Dietz patient had VSRRfollowing ascending aorta replacement, and one (1.8%)Loeys–Dietz patient had VSRR following a failed VSRR at anoutside hospital. Fifty-four (96.4%) were New York HeartAssociation (NYHA) class I preoperatively, while two (3.6%)were class II.

Preoperative sinus diameter was 4.2"0.8 cm (Z-score7.7"2.9), sinotubular junction diameter was 3.1"0.9 cm(Z-score 5.6"3.2) and annular diameter was 2.5"0.4 cm

(Z-score 3.5"2.3). Five (8.9%) had )2q AI on preoperativeechocardiography.

3.2. Operative data

Two patients (3.6%) underwent David I reimplantationwith a straight-tube Dacron graft, 12 (21.4%) had a Yacoubremodeling procedure, and 42 (75.0%) had a reimplantationprocedure using a Valsalva graft. Mean cardiopulmonarybypass and cross-clamp times were 148.9"23.5 and105.0"18.1 min, respectively. The most common prosthe-sis size used was a 24-mm graft in 16 (28.6%) patients.

We routinely inspect for and repair patent foramen ovale(PFO) when present; 33 (58.9%) had a PFO closure at thetime of VSRR. Four (7.1%) had concomitant patent ductusarteriosus (PDA) ligation, three (5.4%) had mitral valverepair, four (7.1%) had atrial septal defect closure, and one(1.8%) had a ventricular septal defect closure. One (1.8%)patient had a hemiarch replacement, one (1.8%) underwentcoronary artery bypass grafting, and one (1.8%) had radio-frequency ablation. Two (3.6%) patients had concomitantaortic valve repair at the time of VSRR.

3.3. Survival and functional class

There was one (1.8%) operative and three (5.4%) latedeaths. Kaplan–Meier survival was 96.4"2.5% at one-,three-, and five-year, and 81.6"10.2% at 10-year (Fig. 1).The lone operative death was a four-year-old female withMarfan syndrome who underwent VSRR and mitral valverepair and experienced massive postoperative bleeding andventricular dysfunction, requiring institution of extracor-poreal membrane oxygenation. The causes of death in thethree late mortalities were type B aortic dissection in two(3.6%) patients and a right coronary artery aneurysm inone (1.8%) patient. Of the 52 patients alive at follow-up,50 (96.2%) were NYHA I and two (3.8%) were NYHA II,postoperatively.

3.4. In-hospital complications

Six (10.7%) patients developed a pneumothorax postop-eratively. Postoperative infectious rates were low. Two(3.6%) children had postoperative pneumonia.

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Fig. 2. Progression of aortic insufficiency and need for aortic valve replace-mentyrepair. Solid lines denote patients requiring surgery. Dashed linesreflect patients with progression of aortic insufficiency currently being fol-lowed. VSRR, value-sparing root replacement; MVR, mitral valve replace-ment; AVR, aortic valve replacement; AV, aortic valve; Preop, preoperative.

Fig. 4. Progression of maximum sinus diameter for patients requiring aorticvalve replacementyrepair. AV, aortic valve; AVR, aortic valve replacement;MVR, mitral valve replacement; Preop, preoperative.

Fig. 3. Progression of annular diameter for patients requiring aorticvalve replacementyrepair. VSRR, valve-sparing root replacement. AV, aorticvalve; AVR, aortic valve replacement; MVR, mitral valve replacement; Preop,preoperative.

3.5. Late complications

Mean follow-up was 5.2 years (range 0–12 years). One 8-year-old female patient with Loeys–Dietz syndrome whounderwent reimplantation within a 24-mm Valsalva graftrequired repair of a distal pseudoaneurysm 29 months post-operatively. Of the 12 patients who underwent remodelingprocedures, four (33.0%) developed )2q AI (Fig. 2). Allfour patients had Marfan syndrome with average max aorticroot diameter of 4.5"1.0 cm (mean Z-score 9.0"1.8 cm).All but one had 0–1q AI preoperatively. All four patientswho developed )2q AI required reoperation: two under-went combined aortic and mitral valve replacement (at 54and 30 months), one underwent aortic valve repair (at96 months), and one had aortic valve replacement alone(at 22 months). Etiology of AI in all cases was dilation ofthe aortic annulus. Progression of aortic annulus and max-imum sinus diameter for the four patients requiring aortic

valve repairyreplacement are shown in Figs. 3 and 4.Preoperative and postoperative maximum sinus and annulardiameters with respective Z-scores are shown in Table 2.No patient developed )2q AI after reimplantation withthe Valsalva graft. Freedom from aortic valve replacementyrepair was 100% after reimplantation vs. 91.7"8.0%, 83.3"10.9%, and 74.1"12.9% at one-, three-, and five-year afterroot remodeling (Ps0.03) (Fig. 5).

No patients suffered thromboembolism or had endocardi-tis at follow-up. No patient had coronary artery anasto-motic dehiscence. At latest follow-up, one child (1.8%)with Loeys–Dietz syndrome developed a right coronaryartery button aneurysm, and one patient with Marfansyndrome (1.8%) developed a type B aortic dissection andis being surveyed radiologically.

3.6. Comment

Marfan syndrome is a well-described aneurysm syndrome,but Loeys–Dietz syndrome has only recently been recog-nized. Loeys–Dietz syndrome results from mutations in thereceptors for transforming growth factor beta (TGF-b) thatlead to abnormal TGF-b signaling w13x. Phenotypic differ-ences between Marfan and Loeys–Dietz syndrome are crit-ical to differential diagnosis, and subsequent operativemanagement. Cardinal manifestations of Marfan syndromeinclude long bone overgrowth, joint laxity, lens dislocation,mitral valve thickening and prolapse, and aortic root aneu-rysm and dissection. On the other hand, Loeys–Dietzpatients manifest a triad consisting of hypertelorism, bifidovula (also cleft palate or both) and arterial tortuosityyaneurysm w13x. What Marfan and Loeys–Dietz patients shareis a predisposition for aneurysm of the aortic root and riskof rupture and dissection, even in children. In our experi-ence, Loeys–Dietz patients are referred for surgical inter-vention at a younger age and smaller aortic root diametersthan patients with Marfan syndrome. Aortic catastrophe

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Table 2. Preoperative and postoperative annular and maximum sinus diameters with corresponding Z-scores

Preoperative Postoperative P-value* Effect size r

Annulus (cm) 2.35"0.36 2.32"0.46 0.37 –0.08Annulus Z-score 3.53"2.33 2.92"2.86 0.22 0.11Maximum sinus (cm) 4.24"0.81 3.23"0.59 -0.001 0.58Maximum sinus Z-score 7.70"2.87 3.08"2.31 -0.001 0.66

*Student’s t-test, preoperative vs. postoperative.

Fig. 5. Freedom from aortic valve replacement/repair. AV, aortic valve.

Table 3. Current guidelines for root replacement at our institution

Maximum Rate ofsinus growthdiameter (cmyyear)(cm)

Marfan syndrome, non-specific )5 )1.0connective tissue disorders,non-connective tissue disordersLoeys–Dietz adults )4 )0.5

Z-score Rate ofgrowth(cmyyear)

Loeys–Dietz type I (-18 years) )3 )0.5Loeys–Dietz type II (-18 years) )4 )0.5

remains a rare but important cause of death in childrenwith aortic aneurysm, and therefore strong considerationshould be given for elective aortic root replacement.

Results following the Bentall operation have been excel-lent and reproducible, and have demonstrated long-termdurability w2–4x. Nevertheless, composite replacement witha mechanical prosthesis necessitates life-long anticoagula-tion and subjects patients to the risk of thromboembolismand prosthetic-valve endocarditis w2–4x. These concerns aswell as the lifestyle implications of anticoagulation havedriven the development of alternative surgical strategiesfor aortic root replacement.

While valve-preservation techniques avoid anticoagula-tion, data regarding long-term durability are lacking inchildren. We reported our early experience with VSRR inchildren in 2005 w14x. In that study, 19 children underwentVSRR. Three children required late aortic valve replace-ment following a remodeling procedure. No patient whohad a reimplantation procedure developed )2q AI orrequired aortic valve replacement. In our initial experi-ence, we used the remodeling technique with scalloped‘Dacron tongues’ because of the theoretical advantage ofpreserving sinuses. Preserving sinuses might minimize leaf-let stress by allowing a more physiological aortic leafletexcursion w15x. However, we observed a trend towards lateannular dilation and subsequent worsening AI, likely due toretained native tissue around the valve hinge points andcommissural posts. In an effort to alleviate annular dilationwe first reinforced the remodeling technique with a Teflonstrip, and then switched to the reimplantation procedurein 2002 when the Valsalva graft, a Dacron graft withprefashioned pseudosinues, became available. We believethat the reimplantation technique using the Valsalva graft

affords the advantage of preserving sinuses, stabilizes theaortic root below the Nadir of the sinuses, and improveshemostasis w4, 9, 11x. In this study, four children developed)2q late AI requiring aortic valve replacementyrepairfollowing remodeling. We are pleased that no patient whohad reimplantation with the Valsalva graft had )2q AI andnone has required aortic valve replacementyrepair. Weperformed reimplantation using a straight Dacron graft intwo patients in this study because the aortic root diameterwas -24 mm, which corresponds to the smallest commer-cially available Valsalva graft. Both of these patients havehad stable repairs with no late AI at follow-up.

Surgeon experience and patient selection are importantfactors and relate to outcomes following VSRR. Our relativecontraindications for VSRR in children include marked leaf-let fenestration and asymmetry; acute aortic dissection inunstable patients; a giant root with marked leaflet irregu-larities; or bicuspid valves with extensive calcification,severe prolapse, andyor marked fenestrations. In our prac-tice, we have proceeded with elective aortic root replace-ment in adults with aortic root diameter of )5.0 cm, rateof aortic root growth )1.0 cmyyear, or with progressivelyworsening AI. Given the greater risk of aortic catastrophein Loeys–Dietz syndrome, we advise earlier aortic rootreplacement in adults when aortic root diameter is)4.0 cm or expands )0.5 cmyyear. For children withsevere craniofacial features (Loeys–Dietz type I) we advisesurgery at an aortic root Z-score )3.0 or an aortic rootexpanding )0.5 cmyyear. For children with mild craniofa-cial features (Loeys–Dietz type II) we recommend surgeryat an aortic root Z-score )4.0 or an aortic root thatexpands )0.5 cmyyear (Table 3).

We recommend that all VSRR patients undergo echocar-diography, CT, or MRI six months postoperatively, andannually thereafter. For Loeys–Dietz syndrome we recom-mend echocardiography every 3–6 months for one year

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after surgery and every six months thereafter, as well asscanning patients from head to pelvis at the time of yearlyCT evaluation.

VSRR is a safe and effective surgical option for themanagement of aortic root aneurysm in children, andeffectively addresses some of the long-term concerns ofmechanical valve replacement. The reimplantation tech-nique with the Valsalva graft produces, in our experience,better results than remodeling by stabilizing the root,improving hemostasis, and preserving sinuses. Althoughlong-term results are still unavailable, mid-term outcomeshave been encouraging.

References

w1x Bentall HH, De Bono A. A technique for complete replacement of theascending aorta. Thorax 1968;23:338–339.

w2x Gott VL, Greene PS, Alejo DE, Cameron DE, Naftel DC, Millar DC,Gillinov AM, Laschinger JC, Pyeritz RE. Replacement of the aortic rootin patients with Marfan’s syndrome. N Engl J Med 1999;340:1307–1313.

w3x Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ,Dietz HC. Aortic root replacement in 271 Marfan patients: a 24-yearexperience. Ann Thorac Surg 2002;73:438–443.

w4x Cameron DE, Alejo DE, Patel ND, Nwakanma LU, Weiss ES, Vricella LA,Dietz HC, Spevak PJ, Williams JA, Bethea BT, Fitton TP, Gott VL. Aorticroot replacement in 372 Marfan patients: evolution of operative repairover 30 years. Ann Thorac Surg 2009;87:1344–1350.

w5x Sarsan MAI, Yacoub M. Remodeling of the aortic valve annulus. J ThoracCardiovasc Surg 1993;105:435–438.

w6x David TE, Feindel CM. An aortic valve-sparing operation for patientswith aortic incompetence and aneurysm of the ascending aorta. JThorac Cardiovasc Surg 1992;103:617–622.

w7x Kallenbach K, Karck M, Pak D, Salcher R, Khaladj N, Leyh R, Hagl C,Haverich A. Decade of aortic valve sparing reimplantation: are wepushing the limits too far? Circulation 2005;112(Suppl I):I-253–I-259.

w8x Settepani F, Szeto WY, Pacini D, De Paulis R, Chiariello L, Di BartolomeoR, Gallotti R, Bavaria JE. Reimplantation valve-sparing aortic rootreplacement in Marfan syndrome using the Valsalva conduit: an inter-continental multicenter study. Ann Thorac Surg 2007;83:S769–S773.

w9x Patel ND, Williams JA, Barreiro CJ, Bethea BT, Fitton TP, Dietz HC,Lima JA, Spevak PJ, Gott VL, Vricella LA, Cameron DE. Valve-sparingaortic root replacement: early experience with the De Paulis Valsalvagraft in 51 patients. Ann Thorac Surg 2006;82:548–553.

w10x Bethea BT, Fitton TP, Alejo DE, Barreiro CJ, Cattaneo SM, Dietz HC,Spevak PJ, Lima JA, Gott VL, Cameron DE. Results of aortic valve-sparing operations: experience with remodeling and reimplantationprocedures in 65 patients. Ann Thorac Surg 2004;78:767–772.

w11x Patel ND, Weiss ES, Alejo DE, Nwakanma LU, Williams JA, Dietz HC,Spevak PJ, Gott VL, Vricella LA, Cameron DE. Aortic root operationsfor Marfan syndrome: a comparison of the Bentall and valve-sparingprocedures. Ann Thorac Surg 2008;85:2003–2011.

w12x Cameron DE, Vricella LA. Valve-sparing aortic root replacement inMarfan syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann2005;8:103–111.

w13x Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H,De Backer JF, Oswald GL, Symoens S, Manouvrier S, Roberts AE, FaravelliF, Greco MA, Pyeritz RE, Milewicz DM, Coucke PJ, Cameron DE,Braverman AC, Byers PH, De Paepe AM, Dietz HC. Aneurysm syndromescaused by mutations in the TGF-b receptor. N Engl J Med 2006;355:788–798.

w14x Vricella LA, Williams JA, Ravekes WJ, Holmes KW, Dietz HC, Gott VL,Cameron DE. Early experience with valve-sparing aortic root replace-ment in children. Ann Thorac Surg 2005;80:1622–1627.

w15x Grande-Allen KJ, Cochran RP, Reinhall PG, Kunzelman KS. Recreationof sinuses is important for sparing the aortic valve: a finite elementstudy. J Thorac Cardiovasc Surg 2000;119:753.

Conference discussion

Dr. C. Schreiber (Munich, Germany): For the benefit of the audience,could you please highlight once again your current selection criteria? In yourmanuscript, you mentioned exclusion criteria, such as leaflet asymmetry,leaflet fenestration, prolapse, or even bicuspid morphology.

Do you believe that the threshold for an attempt to preserve the valvemight come down, provided that surgeons are gaining increasing experience?Recent papers in the adult cohort suggest, for example, that a bicuspidvalve per se should not rule out a valvuloplasty attempt.

Dr. Vricella: This is a very good question. How far can we push repair in apatient? I think that our criteria have evolved to some degree. Initiallybicuspid aortic valves were difficult to include in this particular group.Nowadays, I think we do a fair amount of bicuspid aortic valve repairs.

I think if there are very extensive fenestrations in the area of stress of thevalve, if the valve is bicuspid and stenotic and calcified, or if it requiresmore than just relatively simple techniques, such as shortening of the freemargin of the valve, we would more often go to actually a root replacement,but we do try.

In the technique that we utilize, once we resuspend the commissural posts,then we can really see how we can recreate symmetry within the root andhave a competent valve.

Dr. R. Pretre (Zurich, Switzerland): I would like to go to the very youngpatients. We sometimes see nine-year-old kids with already a very hugelydilated aortic root. How do you manage that? Do you already go to areplacement with a very small graft, or do you wait until they are 14, 15?What is the risk to wait here?

Dr. Vricella: Sure. I think you can stratify the risk based on the etiologyof the disease. So, in patients, for example, with Marfan syndrome, theliterature shows that it is relatively safe to wait until they almost meetadult criteria. And there are actually retrospective reviews of patients thatwere observed during childhood, and in the incidence of rupture, dissectionis very low.

So we would actually utilize adult criteria, let’s say, in the nine-year-oldchild with Marfan syndrome. We would probably wait until we reach 4.5–5 cm in maximal diameter or if there is de novo aortic regurgitation. Inpatients who have more aggressive disease, such as Loeys–Dietz syndrome,for example, we actually would lower that threshold. Not as much as byZ-scores, but we would lower it to 4 cm.

As you know, there is Loeys–Dietz syndrome type I and II. Type I, which isthe more aggressive form, is characterized by severe craniofacial features.In those cases, we actually go down even to 3.5 or 3 cm because there hasbeen incidence of rupture, dissection actually, as early as nine months ofage. So in those cases, we would utilize a lower threshold.

Otherwise, for patients who encompass half of our experience, the Marfanpatients, we will wait a few years. But there is a substantial number ofpatients that are in the six- to eight-year-old range and for whom thisquestion is very pertinent.

Dr. P. del Nido (Boston, MA, USA): I have just have one quick follow-upquestion to what Dr. Pretre just asked.

Do you vary where you stop on your distal anastomosis depending on thediagnosis or the age of the patient?

Dr. Vricella: Yes. That is a very good question, because with Marfanpatients, for example, it is rare for us to do the entire arch if it is of normalsize. Not a lot of these patients will actually present with late archdilatation. You worry more about type B dissection with the descendingaorta.

In patients with Loeys–Dietz syndrome, we are thinking about doing thisbecause those patients do develop arch enlargement early on. It is a muchmore malignant syndrome, and they also develop pseudoaneurysms. So, itis tempting to actually do the remainder of the arch.

The problem is that if you have, for example, an eight-year-old child withan arch that is of normal size, by replacing it you expose the patient to asignificant amount, I think, of additional morbidity associated with archreplacement and now have several suture lines that are at risk of developingpseudoaneurysms.

So, I think the jury is still out, but it is one thing that we are clearlyconsidering for patients with Loeys–Dietz syndrome.

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