Surgical Management of Supravalvular Aortic Stenosis: Does Brom Three-Patch Technique Provide Superior Results?

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1 Surgical Management of Supravalvular Aortic Stenosis: Does Brom Three-Patch Technique Provide Superior Results? Olivier Metton, MD, Walid Ben Ali, MD, Davide Calvaruso, MD, Damien Bonnet, MD, PhD, Daniel Sidi, MD, PhD, Olivier Raisky, MD, PhD, and Pascal R. Vouhé, MD, PhD Departments of Pediatric Cardiac Surgery, and Pediatric Cardiology, University Paris Descartes and Sick Children Hospital, Paris, France Background. Various surgical techniques have been proposed to repair supravalvular aortic stenosis. However, the optimal approach remains to be determined. The present study was undertaken to evaluate the results of surgery for supravalvular aortic stenosis and to compare the symmetric three-patch repair (Brom technique) with other surgical reconstructions. Methods. Between 1995 and 2007, 34 patients with supravalvular aortic stenosis underwent surgery. Fourteen patients (41%) had Williams syndrome. Supravalvular stenosis was discrete in 24 patients (71%) and diffuse in 10 (29%). Eight patients (23%) underwent one-patch repair; 3 patients (9%) had inverted bifurcated patch enlargement (Doty repair) and 23 patients (68%) had symmetric repair (Brom three-patch technique). Follow-up was complete and ranged from 6 months to 12 years (mean, 5.8 years). Results. There was one early and no late deaths. One patient (with one-patch repair) underwent reoperation for residual obstruction. At last follow-up, left ventricle to aorta peak gradient was mm Hg after onepatch repair, 30 9 mm Hg after Doty operation, and mm Hg after symmetric Brom procedure. Brom repair was associated with a low incidence of residual obstruction (peak gradient > 40 mm Hg) (2 of 22; 9.1%) and moderate aortic insufficiency (1 of 22; 4.5%). Conclusions. Brom three-patch repair provides symmetric reconstruction of the aortic root in patients with supravalvular aortic stenosis. This may lead to improved midterm results in terms of relief of the obstruction and incidence of aortic insufficiency. (Ann Thorac Surg 2009;88:588 93) 2009 by The Society of Thoracic Surgeons Supravalvular aortic stenosis is an uncommon congenital malformation. Supravalvular aortic stenosis represents an important feature of Williams syndrome but may also occur as familial forms or sporadic cases. The underlying cause is now known to be a spontaneous or inherited mutation of the elastin gene on chromosome 7. The resulting thickening of the aortic wall is characteristically localized at the sinotubular junction but it may extend into the ascending aorta, the aortic arch, and the arch vessels. Supravalvular aortic stenosis may also be part of a generalized arteriopathy involving both the systemic and pulmonary arterial systems. The first successful surgical repair was achieved in 1956, using enlargement of the noncoronary sinus across the stenotic area by a single diamond-shaped prosthetic patch [1]. This technique became the most commonly performed procedure to treat the anomaly. Although the obstruction is usually relieved adequately, the normal configuration of the aortic root is not restored. An effort to provide a more anatomic repair of the aortic root was reported by Doty and colleagues in 1977 [2]. An inverted Accepted for publication April 24, Address correspondence to Dr Vouhé, Chirurgie Cardiaque Pédiatrique, Hôpital Necker Enfants Malades, 149 rue de Sèvres, Paris, 75015, France; pascal.vouhe@nck.aphp.fr. bifurcated patch was inserted into the noncoronary sinus and the right coronary sinus. To achieve symmetric enlargement of the aortic root, Brom introduced aortoplasty by the three-patch technique. This procedure was used by Brom as early as 1978 [3, 4]. However, the procedure was popularized only more recently and several modifications have been described [5 7]. Because of the rarity of the disease, limited data are available. Published surgical series report relatively small numbers of patients, operated upon over long periods of time [4, 8 12]. Few allow direct comparison of the various surgical techniques and the superiority of one of them has not been clearly documented so far. Brom repair was introduced in our unit in Since that date other techniques of reconstruction were used as well. The present study was undertaken to evaluate the midterm results obtained using various techniques to repair supravalvular aortic stenosis. Patients and Methods Patient Population To find the reports of patients who underwent surgery for supravalvular aortic stenosis at Sick Children Hospital, Paris, the cardiology and cardiac surgery databases 2009 by The Society of Thoracic Surgeons /09/$36.00 Published by Elsevier Inc doi: /j.athoracsur

2 Ann Thorac Surg METTON ET AL 2009;88: SUPRAVALVULAR AORTIC STENOSIS 589 were reviewed. Patients with acquired postsurgical supravalvular aortic obstruction were excluded. Permission to perform health records review was obtained from the Paris Descartes University Ethics Committee. The need for individual consent was waived. Between January 1995 and June 2007, 34 patients underwent surgery for congenital supravalvular aortic stenosis. Twenty-two (65%) of the patients were male and 12 (35%) were female. The age at the time of operation ranged between 2 months and 24 years, with a mean of years. In 14 patients (41%), a diagnosis of Willams syndrome was made by a geneticist and supported, in the more recent period, by fluorescence in situ hybridization analysis of a deletion on chromosome 7q The remaining 20 patients (69%) were classified as sporadic. A harsh systolic murmur was present in all patients and represented the most common cause for initial consultation. The other clinical presentations included a history of syncopal episodes, exercise intolerance, and intermittent angina. Upon clinical presentation, 24 patients (71%) were in New York Heart Association (NYHA) functional class I, 5 in class II, and 5 in class III. The supravalvular narrowing was discrete, localized to the sinotubular junction in 24 patients (71%); in 10 patients (29%) the stenosis was diffuse, involving the ascending aorta (3 cases) or the ascending and transverse aorta (7 cases). The left ventricle to aorta peak gradient (as evaluated by pulsed Doppler echocardiography) ranged between 70 and 145 mm Hg, with a mean of mm Hg. The aortic valve was bicuspid in 4 cases. There were 5 patients with valvular aortic stenosis and 3 with muscular subaortic obstruction. No patient had more than trivial aortic regurgitation. Echocardiographic evidence of moderate left ventricular hypertrophy was present in 20 cases (69%); severe hypertrophy was noted in 14 patients. The mean preoperative left ventricular ejection fraction was Branch pulmonary arterial stenoses were present in 15 patients (44%). Coronary arterial involvement was demonstrated in 3 patients; severe left main coronary artery stenosis was diagnosed preoperatively in 1 patient; in the 2 other patients, restricted access to one of the coronary arteries was observed intraoperatively. Systemic arterial stenoses were noted in 4 patients and involved the renal arteries (2 cases) and mesenteric vessels (2 cases). One patient had a small muscular ventricular septal defect. Four patients had previously undergone cardiovascular operations: coarctation repair (3 patients) and aortic valvotomy (1 patient). Operative Techniques All repairs were performed through a median sternotomy using cardiopulmonary bypass and cardioplegic arrest. Concomitant repair of the arch was achieved under deep hypothermic circulatory arrest with selective cerebral perfusion. Various surgical techniques were used. One of the authors (PRV) performed 25 operations (73% of all operations), including 4 (50%) one-patch repairs, 2 (66%) Doty operations, and 19 (83%) Brom procedures. ONE-PATCH REPAIR. Eight patients (23%) underwent a onepatch repair. A diamond-shaped prosthetic patch was inserted in the ascending aorta after longitudinal incision through the stenotic area extending into the noncoronary sinus. BIFURCATED-PATCH REPAIR (DOTY TECHNIQUE). In 3 patients (9%), a bifurcated patch plasty, as described by Doty [2], was used. An inverted Y-shaped incision was made into the ascending aorta down into the noncoronary sinus and the right coronary sinus to the left of the right coronary ostium. SYMMETRIC THREE-PATCH REPAIR (BROM TECHNIQUE). The symmetric three-patch technique as reported by Brom [3], was used in 23 patients (68%) The aorta was transected immediately above the area of maximal stenosis. A longitudinal incision was made into each of the three sinuses (central in the noncoronary sinus, to the left of the right coronary ostium and to the right of the left coronary ostium). Each incision was carried out approximately halfway down into the respective sinus or deeper if required. Three separate prosthetic patches (heterologous pericardium in most cases) were tailored to provide symmetric reconstruction of the aortic root and inserted accordingly. The reconstructed aortic root was anastomosed end to end to the distal ascending aorta; in 5 patients an additional anterior pericardial patch was used to enlarge the ascending aorta. ASSOCIATED PROCEDURES. A restricted access to coronary arteries was documented in 3 cases: left main coronary artery stenosis in one, abnormal adherence of a cusp to the sinotubular ridge limiting coronary blood flow in 2 patients (left coronary and right coronary once each). Right coronary ostial stenosis was relieved by direct excision. The 2 patients with left coronary obstruction underwent patch coronary angioplasty using a separate saphenous vein patch [13]. Patch enlargement of the transverse aortic arch was carried out in 7 patients. Other associated procedures included aortic valvotomy (5 cases), subaortic myectomy (3 cases), and patch enlargement of pulmonary arterial Table 1. Intraoperative Data Variable One-patch Repair Doty Repair Brom Repair (8 patients) (3 patients) (23 patients) Arch repair 3 (37.5%) 0 4 (17%) Coronary repair (13%) Aortic valvotomy 2 (25%) 1 (33%) 2 (9%) Subaortic myectomy 1 (12.5%) 0 2 (9%) Pulmonary artery plasty 1 (12.5%) 0 4 (17%) Perfusion time (mn) Cross-clamp time (mn)

3 590 METTON ET AL Ann Thorac Surg SUPRAVALVULAR AORTIC STENOSIS 2009;88: stenoses (5 cases). Additional intraoperative details are given in Table 1. Follow-Up and Data Analysis Follow-up data were obtained during a 6-month closing interval (January 2008 to June 2008) by physician or patient contact when the patients were not followed up in our hospital. Information was obtained for all patients. Echocardiogram reports were obtained at the time of last follow-up in all cases. Peak left ventricular-aortic gradient was evaluated and aortic regurgitation was graded as none-trivial, mild, moderate, or severe. Data were described as frequencies, medians with ranges, and means with standard deviations. Crude ratios were given with 70% confidence limits. Continuous variables were compared using the independent t test or Mann-Whitney U test when data were not normally distributed. The Pearson 2 or Fisher exact test were used to determine differences when variables were expressed by dichotomous values. A p value less than 0.05 was set as the level of statistical significance. All analyses were performed using SPSS for Windows version 16.0 (SPSS Inc, Chicago, IL). Table 2. Risk Factors for Early Residual Obstruction (Gradient 40 mm Hg) Risk Factors p Value Sex Williams syndrome Valvular aortic stenosis Subaortic stenosis Bicuspid aortic valve Diffuse form Pulmonary artery stenoses Type of repair Aortic valvotomy Subaortic myectomy Results Early Results There was one hospital death (2.9% early mortality rate, 70% confidence limits: 0.4% to 9.4%). A 3-month-old boy had diffuse supravalvular aortic stenosis with severe stenosis of the left main coronary artery. He underwent Brom repair and patch angioplasty of the left coronary artery and enlargement of the aortic arch; after repair, he had diffuse signs of myocardial ischemia with left ventricular dysfunction and could not be weaned from cardiopulmonary bypass. For operative survivors, the mean duration of ventilatory support was hours and the mean intensive care unit stay was days. The mean left ventricle to aorta peak systolic gradient at hospital discharge was mm Hg; it was significantly reduced compared with preoperative values (p 0.001). The mean peak gradient at hospital discharge was mm Hg after one-patch repair, mm Hg after Doty operation, and mm Hg in patients with symmetric Brom procedure (Fig 1). Eight patients had an early residual gradient equal or superior to 40 mm Hg: 1 after three-patch repair, 1 after Doty procedure, and 6 after one-patch repair (p 0.001). Potential risk factors for residual obstruction (gradient 40 mm Hg) were investigated (Table 2). Bicuspid aortic valve and type of operation were identified as incremental risk factors. At hospital discharge, aortic regurgitation was absent or trivial in 18 patients (55%), mild in 12 (33%), and moderate in 3 (12%). None of the patients who underwent Brom repair had more than mild aortic regurgitation. Late Results The mean duration of follow-up was years (range, 6 months to 12 years). The mean duration of follow-up was months after one-patch repair, Table 3. Risk Factors for Late Residual Obstruction (Gradient 40 mm Hg) Risk Factors p Value Fig 1. Peak pressure gradients according to type of repair, preoperatively, immediately after surgery and at last follow-up. Gradients are expressed as means with one standard deviation. Mean follow-up was years following one-patch repair (Œ-Œ), years after Doty procedure ( - ), and years after Brom repair ( - ). Sex Williams syndrome Valvular aortic stenosis Subaortic stenosis Bicuspid aortic valve Diffuse form Pulmonary artery stenoses Type of repair Aortic valvotomy Subaortic myectomy 1.000

4 Ann Thorac Surg METTON ET AL 2009;88: SUPRAVALVULAR AORTIC STENOSIS 591 Fig 2. Incidence and severity of aortic regurgitation at last followup, according to type of repair. After Brom repair, only one patient had moderate insufficiency. (Black moderate; grey mild; white 0/trivial.) months after Doty procedure, and months after Brom repair. There was no late mortality. One patient underwent reoperation: he had residual obstruction after an initial one-patch repair, he underwent three-patch repair 20 months after the first operation, and at last evaluation, 27 months after reoperation, the peak gradient was 10 mm Hg. At last follow-up, 28 patients (85%) were free of symptoms, 3 patients were in NYHA functional class II, one had a syncopal episode of unknown cause, and one had a history of chest pain at exercise without evidence of coronary obstruction. The mean left ventricle to aorta peak gradient at last follow-up was mm Hg. The peak gradient was mm Hg after one-patch repair, 30 9mmHg after Doty operation, and mm Hg after symmetric Brom procedure (Fig 1). Nine patients had a late residual gradient equal or superior to 40 mm Hg: 2 after symmetric Brom repair, 6 after one-patch repair, and one after Doty operation (p 0.002). Factors potentially associated with an increased risk of late residual obstruction (peak gradient 40 mm Hg) were investigated (Table 3). Bicuspid aortic valve, Williams syndrome, pulmonary arterial stenoses, and type of repair were identified as incremental risk factors. At last follow-up, aortic regurgitation was absent or trivial in 20 patients (61%), mild in 8 (24%), and moderate in 5 (15%). The incidence and severity of aortic insufficiency according to the type of operation is shown in Figure 2. After Brom repair, only 1 patient (4.5%) had more than mild aortic insufficiency. Comment Elastin Arteriopathy Supravalvular aortic stenosis is not a simple lesion. The underlying elastin arteriopathy is a generalized disease of both pulmonary and systemic arteries. Elastin arteriopathy is a consequence of microdeletion involving the elastin gene on chromosome 7q Individuals affected with elastin arteriopathy exhibit an overall reduced arterial elastin content. Reduced elasticity of the great arteries increases shear stress in the vascular wall, and provokes smooth muscle hypertrophy and increased collagen deposition, resulting in marked medial thickening. The resulting luminal obstruction ranges from localized stenosis of the supravalvular aortic area to diffuse narrowing extending into the arch and the descending aorta, including the origins of the head and neck vessels and coronary arteries. Other large elastic arteries (including the pulmonary arteries) may also be affected [14]. Regarding the natural history of the disease, it has long been established that the severity of pulmonary arterial involvement decreases spontaneously throughout childhood and adolescence. It has been shown recently that systemic arterial lesions may also gradually regress over time [12]. It is therefore advocated to observe a wait-andsee approach in patients with moderate lesions and to indicate surgical repair only in children with severe and symptomatic lesions. Surgery for Supravalvular Aortic Stenosis The hallmark of elastin arteriopathy is supravalvular aortic stenosis at the sinotubular junction. The geometry of the sinotubular junction is important for left ventricular outflow tract flow dynamics. In normal hearts, the aortic root including the flexible sinotubular junction expands during systole, straightening the leaflet free edges and maintaining a constant strain in order to minimize fatigue stress. In supravalvular aortic stenosis, the sinotubular junction is rigid and there is a mismatch between the free edge of the leaflets and the corresponding part of the sinotubular junction [15]. Normal mechanics of the aortic root are impeded and premature degeneration of the leaflets may be promoted. The first and main goal of surgical treatment is to relieve left ventricular outflow tract obstruction; this can be achieved using nonsymmetric (one patch or inverted Y-shaped patch) as well as symmetric three-patch repair. In an attempt to optimize results, consideration should, however, be given also to restoring the physiologic anatomy of the aortic root. Diamond-shaped single patch repair does not restore symmetric geometry of the sinotubular junction. The inverted Y-shaped patch repair represents an attempt to improve geometry but leaves potential stenosis above the left coronary sinus. Only the three-patch technique can provide symmetric repair of the aortic root. Nevertheless, the superiority of this technique has not been clearly documented so far. Series using predominantly either diamond-shaped patch repair [8, 11] or inverted Y-shaped patch repair [10 12] have reported satisfactory early and late results. However, long-term results are often not optimal; moderate pressure gradients and aortic valve regurgitation persist or recur in up to 50% of patients. Comparative studies are very rare. Hazekamp and colleagues [4] reported their experience comparing three-patch repair (13 patients) with one-sinus or two-sinuses reconstruction (16 patients); they did not find a significant difference

5 592 METTON ET AL Ann Thorac Surg SUPRAVALVULAR AORTIC STENOSIS 2009;88: in long-term gradients or need for reoperation. In the Boston group experience [9], one-patch repair (34 patients) was compared to multiple-sinuses reconstruction (41 patients); survival and freedom from reoperation did not reach statistical significance, but there were no late deaths, less reoperations and lower long-term pressure gradients in patients with two- or three-sinuses repairs. In the current series, satisfactory results were obtained using all three techniques, but three-patch repair was associated with a lower incidence of residual left ventricular outflow tract obstruction and moderate aortic insufficiency. This may provide a superiority of this approach on the long term. Three-patch repair using separate patches of autologous or heterologous pericardium has therefore become our procedure of choice. Autologous pericardium, treated extemporaneously with glutaraldehyde, is an attractive material because of its growth potential; however, because of a potential risk of aneurismal dilatation with progressive aortic insufficiency, this material is used only in small infants. In most patients, heterologous pericardium is preferred. One potential drawback of Brom repair is aortic regurgitation due to oversizing of the reconstructed sinuses. Appropriate tailoring of the patches, according to the age-normalized circumference of the aortic root, should prevent this complication. When necessary, an additional pericardial patch should be inserted to enlarge the ascending aorta. Modification of the Brom technique by adequate tailoring of the distal aorta is appealing because it avoids any patch [5]. However, it requires extensive mobilization of the arch and carries a potential risk of compression of the left main bronchus and left pulmonary artery; it was not used in our series. The presence of a bicuspid aortic valve warrants adequate tailoring of the patches but does not preclude symmetric repair. In the current series, associated bicuspid aortic valve or valvular stenosis were not incremental risk factors for unsatisfactory outcome. Associated lesions involving the coronary arteries, the aortic arch, or the brachiocephalic vessels must be relieved using appropriate techniques [16]. In patients with diffuse supravalvular aortic stenosis, the ascending aorta and transverse arch should be enlarged using a prosthetic patch inserted in the concavity of the arch. The late outcome of diffuse supravalvular aortic stenosis is, somewhat surprisingly, similar to that of the localized form, although the early risk is probably increased [14]; the only death in our series had diffuse supravalvular stenosis. None of the residual obstructions were located at the level of the aortic arch, beyond the ascending aorta. Coronary arterial obstructions may be life-threatening and must be relieved. It is essential to look for coronary lesions before surgery and to inspect the coronary ostia at the time of aortic repair. Ostial obstructions (often due to abnormal adhesions of the valvar leaflets to the sinotubular ridge) can be relieved by simple excision; involvement of the proximal coronary arteries needs separate patch enlargement [13]. Even if the natural course of pulmonary arterial lesions favors spontaneous regression it is recommended to repair, during the same operation, severe central pulmonary artery stenoses amenable to adequate surgical correction, in order to decrease postoperative right ventricular overload and reduce the risk of right ventricular failure [14]. Limitations of the Study The results of the current study must be interpreted with caution for several reasons. The study population is small because of the rarity of the malformation and, therefore, the number of patients undergoing each type of repair is small. Most nonsymmetric repairs were performed before Only 3 patients underwent Doty repair; the results after this approach were satisfactory and may compare similarly in the long-term with the results of the Brom procedure. The overall duration of follow-up remains short, particularly after symmetric reconstruction Most Brom operations were carried out by the same senior surgeon. This may represent a major limitation. However, careful evaluations of the 25 patients operated upon by the same surgeon were carried out. Similar results were obtained. At last follow-up, the mean peak gradient was lower after Brom repair compared with one patch-repair (8 15 mm Hg vs mm Hg). The very small number of patients undergoing Doty repair, does not allow to draw out conclusions. Conclusions The current study shows that the midterm postoperative outcome of children with supravalvular aortic stenosis is satisfactory, even in patients with diffuse forms and associated lesions. The results after three-patch repair are superior to those of one-patch reconstruction in terms of residual obstruction and development of aortic insufficiency. This finding is sensible because three-patch repair reconstructs a symmetric aortic root and should restore a normal physiology. However, further experience and longer follow-up are mandatory to confirm these preliminary data and the current study does not provide adequate evaluation of the Doty technique. We gratefully acknowledge the secretarial assistance of Ms Corinne Pasquet. References 1. McGoon DC, Mankin HT, Vlad P, Kirklin JW. The surgical treatment of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1961;92: Doty DB, Polansky DB, Jenson CB. Supravalvular aortic stenosis: repair by extended aortoplasty. J Thorac Cardiovasc Surg 1977;74: Brom AG. Obstruction of the left ventricular outflow tract. In: Khonsari S, ed. Cardiac surgery: safeguards and pitfalls in operative technique. Rockville, MD: Aspen Publishers; 1988: Hazekamp MG, Kappetein AP, Schoof PH, et al. Brom s three-patch technique for repair of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1999;118:252 8.

6 Ann Thorac Surg METTON ET AL 2009;88: SUPRAVALVULAR AORTIC STENOSIS Myers JL, Waldhausen JA, Cyran CE, Gleason MM, Weber HS, Baylen BG. Results of surgical repair of congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1993;105: Steinberg JB, Delius RE, Behrendt DM. Supravalvular aortic stenosis: a modification of extended aortoplasty. Ann Thorac Surg 1998;65: Al-Halees Z, Prabhkar G, Galal O. Reconstruction of supravalvar aortic stenosis with autologous pulmonary artery. Ann Thorac Surg 1998;65: van Son JAM, Danielson GK, Puga FJ, et al. Supravalvular aortic stenosis. Long-term results of surgical treatment. J Thorac Cardiovasc Surg 1994;107: Stamm C, Kreutzer C, Zurakowski D, et al. Forty-one years of surgical experience with congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg 1999;118: McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, Hanley FL. Issues and outcomes in the management os supravalvar aortic stenosis. Ann Thorac Surg 2000;69: INVITED COMMENTARY Supravalvar aortic stenosis is a rare lesion. In the early years of congenital cardiac surgery, it was managed in the same way as any circumferential stenosis by using a simple single-patch plasty technique. Doty contributed an important advance with the introduction of the inverted bifurcated patch. Although Doty s technique effectively enlarges the circumference of the sinotubular ridge, which is usually the site of most severe stenosis, it does not provide a symmetric reconstruction or enlargement of the left coronary sinus. Recognizing these deficiencies as well as the importance of restriction of coronary blood flow into the left coronary sinus has led many centers during the last 10 to 15 years to begin applying the symmetric 3-patch technique originally described by Brom. The authors of the article published in this issue [1] are correct in stating that there is little information available to clarify the advantages or disadvantages of the Brom technique relative to the more traditional Doty technique. After their analysis, the authors strongly endorse the 3- patch technique. Their conclusions are similar to those drawn from a series of 75 patients managed at Children s Hospital Boston during a 41-year experience. Multiple sinus reconstruction appears to result in superior hemodynamics and is associated with reduction in both mortality rate and need for reoperation. One limitation of the article by Metton and colleagues [1] is the same senior surgeon did most of the Brom operations. It is also not clear which criteria were used to select the 1- vs 2- vs 3-patch technique. The limitations 11. Brown JW, Ruzmetov M, Vijay P, Turrentine MW. Surgical repair of congenital supravalvular aortic stenosis in children. Eur J Cardiothorac Surg 2002;21: Hickey EJ, Jung G, Williams WG, et al. Congenital supravalvular aortic stenosis: defining surgical and nonsurgical outcomes. Ann Thorac Surg 2008;86: Borgoënd E, Raisky O, Degandt A, Tamisier D, Sidi D, Vouhé P. Myocardial revascularization in infants and children by means of coronary artery proximal patch arterioplasty or bypass grafting: a single-institution experience. J Thorac Cardiovasc Surg 2008;136: Stamm C, Friehs I, Ho SY, Moran AM, Jonas RA, del Nido P. Congenital supravalvar aortic stenosis: a simple lesion? Eur J Cardiothorac Surg 2001;19: Stamm C, Li j, Ho SY, Redington AN, Anderson RH. The aortic root in supravalvular aortic stenosis: the potential surgical relevance of morphologic findings. J Thorac Cardiovasc Surg 1997;114: Prêtre R, Arbenz U, Vogt PR, Turina MI. Application of successive principles of repair to correct supravalvular aortic stenosis. Ann Thorac Surg 1999;67: statement of the article suggests that this may have been mainly surgeon-dependent, which considerably weakens the findings of the study. However, a subsequent analysis undertaken by the authors looking only at 25 patients operated on by the same surgeon also suggests superiority of the Brom repair. Considerable progress has been made in understanding the genetic basis of the elastin arteriopathy that causes supravalvar aortic stenosis. This lesion is deceptively complex and involves abnormalities of coronary perfusion as well as left ventricular hypertension and hypertrophy. Nevertheless the long-term results of surgical repair are surprisingly satisfactory. The article by Metton and colleagues [1] provides further evidence that symmetric reconstruction with 3 patches is likely to be the optimal method of repair for most patients. Richard Jonas, MD Division of Cardiac Surgery Children s National Medical Center 111 Michigan Ave NW Washington, DC rjonas@cnmc.org Reference 1. Metton O, Ben Ali W, Calvaruso D, et al. Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results? Ann Thorac Surg 2009; 88: by The Society of Thoracic Surgeons /09/$36.00 Published by Elsevier Inc doi: /j.athoracsur