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Ann Thorac Surg 2000;69:562-567
© 2000 The Society of Thoracic Surgeons

Original Articles

Issues and outcomes in the management of supravalvar aortic stenosis

Doff B. McElhinney, MDa, Edwin Petrossian, MDa, Wayne Tworetzky, MDa, Norman H. Silverman, MDa, Frank L. Hanley, MDa

a Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of California, San Francisco, California, USA

Address reprint requests to Dr McElhinney, Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Rm 9557, Philadelphia, PA 19104
e-mail: mcelhinney{at}email.chop.edu


   Abstract
Top
 Abstract
Introduction
 Patients and methods
 Results
 Comment
 References
 
Background. Supravalvar stenosis of the aorta is an uncommon congenital cardiac anomaly that involves not only the supravalvar aorta but the entire aortic root. Despite considerable attention to the importance of maintaining the integrity of the aortic root during supravalvar reconstruction, there has been little focus on the management of other components of the aortic root and left ventricular outflow tract, including the aortic valve, subvalvar region, and coronary arteries.

Methods. We reviewed the records of 36 consecutive patients with supravalvar aortic stenosis who underwent repair from 1992–1998 (median age, 4 years). Discrete stenosis was present in 29 patients, whereas the remaining 7 had the diffuse form of the disease. Associated anomalies of the aortic root and adjacent structures were present in 23 patients. The median pressure gradient across the left ventricular outflow tract was 70 mm Hg. Supravalvar stenosis was relieved by extended aortoplasty with a Y-shaped patch in 18 patients, resection of the stenotic segment of ascending aorta at the sinotubular junction with end-to-end anastomosis of the ascending aorta in 7, the Ross procedure in 4, and other techniques in 7. Additional procedures included aortic valvuloplasty in 10 patients, resection of subvalvar stenosis in 11, and procedures on the coronary arteries in 2.

Results. There was 1 perioperative death, and no reoperations or other significant complications. During follow-up (median 33 months), there were no deaths and 3 reoperations for replacement of the aortic valve with a pulmonary autograft (n = 1) or mechanical prosthesis (n = 2). The median pressure gradient across the left ventricular outflow tract was 10 mm Hg.

Conclusions. In patients with supravalvar aortic stenosis, abnormalities of the aortic valve, subaortic region, and coronary arteries are frequently present as well. Management of these issues is as critical to the long-term outcome of these patients as reconstruction of the supravalvar aorta. Aggressive valvuloplasty may help decrease the incidence of late aortic valve replacement, whereas the Ross procedure may be a preferable approach in some patients with complex outflow tract obstruction.


   Introduction
Top
 Abstract
 Introduction
Patients and methods
 Results
 Comment
 References
 
Supravalvar aortic stenosis is a relatively uncommon form of congenital obstruction of the systemic outflow tract. As Stamm and associates have shown recently [1], this is not simply a disease of the supravalvar aorta, but of the entire aortic root. Recognition of this fact by surgeons is illustrated in the evolution of operative approaches to this lesion, which has seen an increasing emphasis on reconstructive techniques that preserve the integrity of the aortic root [2–7]. Despite, the focus on techniques of supravalvar reconstruction, relatively little attention has been paid to the issues of the aortic valve, subvalvar region, and coronary arteries in the management of this anomaly. The aortic valve is frequently abnormal and dysfunctional in patients with supravalvar aortic stenosis, and procedures on the valve are performed at the time of repair of supravalvar aortic stenosis in a substantial proportion of patients [3–5]. Moreover, dysfunction of the aortic valve is the most common reason for reoperation in these patients [3–5], and in at least one study, associated aortic valvar pathology (stenosis or regurgitation) was found to be the only significant predictor of decreased long-term survival [5]. Obstruction of the subvalvar outflow tract is also an important problem in a number of patients, as are structural and physiologic abnormalities of the coronary arteries. Despite the central role that valvar pathology plays in the treatment of supravalvar aortic stenosis, little attention is given to this issue [8], or to those of concomitant management of subvalvar obstruction or coronary arterial pathology. In this report, we present the results of a review of our surgical experience with supravalvar aortic stenosis over the past 6 years, with a focus on the importance of the entire aortic root and adjacent structures in this condition.


   Patients and methods
Top
 Abstract
 Introduction
 Patients and methods
Results
 Comment
 References
 
Patients
From 1992 through 1998, 36 patients with supravalvar aortic stenosis underwent repair. The median age was 4 years, with a range of 2 months to 33 years. Eight patients had undergone a total of 10 prior operations, including repair of supravalvar aortic stenosis in 3, aortic valvuloplasty in 4, and resection of subvalvar left ventricular outflow tract obstruction in 3. The supravalvar aortic stenosis was focal (discrete) in 29 patients and diffuse in 7. Twelve patients had valvar aortic stenosis, and 11 had aortic regurgitation of mild or greater degree (mild in 4, moderate in 6, and severe in 1). A bileaflet aortic valve was present in 14 patients, and subvalvar obstruction was present in 11. Most patients had some degree of dilatation of the aortic sinuses. Three patients had significant abnormalities of the coronary arteries or coronary artery flow, including diffuse stenosis of the left main coronary artery (Fig 1), stenosis of the left coronary ostium, and isolation of the right coronary artery as a result of complete fusion of the right coronary leaflet to the supravalvar ridge. The total gradient across the left ventricular outflow tract (from subvalvar to supravalvar regions) ranged from 25 to 150 mm Hg (median, 70 mm Hg), and across the supravalvar stenosis from 20 to 125 mm Hg (median, 50 mm Hg) in patients in whom it could be assessed reliably. Williams syndrome was present in 12 patients, 10 of whom had stenosis of the pulmonary arteries, mild in 3 and significant in 7. Other associated anomalies included coarctation of the aorta and atrial septal defect, each of which were found in 1 patient.



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  		Fig 1. Aortic root angiogram in a 5-month-old patient, demonstrating discrete supravalvar aortic stenosis and diffuse stenosis of the left main coronary artery.

 
Operative procedures
Procedures to repair supravalvar stenosis
Supravalvar stenosis was repaired using one of several techniques. In 18 patients, extended aortoplasty with a patch in the shape of an inverted Y was performed. A longitudinal aortotomy was extended from the nonstenotic segment of ascending aorta distal to the supravalvar stenosis, deep into the right and noncoronary sinuses of Valsalva. The supravalvar ridge was excised carefully, and procedures on the aortic valve, root, and subvalvar region were performed if necessary (described below). The aorta was then augmented with a Y-shaped patch of Dacron vascular graft using continuous nonabsorbable monofilament suture, in the style originally described by Doty and associates [2].

In 7 patients, supravalvar aortic stenosis was repaired in a manner similar to the techniques described by Myers and colleagues [6] and by Chard and Cartmill [7]. The ascending aorta was transected distal to the stenotic segment, which was excised after evaluating the aortic valve. The proximal rim of the excision was carried below the commissural apices of the aortic valve, with the maximum amount of aortic wall tissue excised from the sinuses of Valsalva as possible without impinging on the orifices of the coronary arteries. If necessary, procedures on the aortic valve and subvalvar region were then carried out (see below). The distal edge of ascending aorta was scalloped and anastomosed directly to the proximal rim with continuous nonabsorbable monofilament sutures.

In 4 patients, a Ross or Ross-Konno procedure was performed, as described previously [9]. In all of these, the segment of ascending aorta distal to the pulmonary autograft was augmented with a single triangular patch of aortic allograft tissue.

Other techniques used to relieve supravalvar stenosis included patch augmentation of the root and ascending aorta (n = 5, 4 with diffuse disease), resection of the supravalvar ridge without transection or augmentation of the ascending aorta (n = 1), and patch augmentation in conjunction with repair of aortic coarctation using a technique of end-to-side anastomosis of the descending aorta to the ascending aorta/proximal arch (n = 1).

Procedures on the left ventricular outflow tract and coronary arteries
In addition to the 4 patients who underwent a Ross or Ross-Konno operation, 16 procedures were performed on the aortic valve in 10 patients. Thus, procedures on the aortic valve were performed in 14 patients (39%). These included, in addition to the Ross procedure, mobilization of restricted leaflets (commissurotomy and detachment of leaflets partially fused to the supravalvar ridge) in 8 patients, thinning of the leaflets in 5, resuspension of 1 or more leaflets in 2, augmentation of a leaflet in 1, and detachment of the right coronary leaflet from the supravalvar ridge to which it was completely fused in 1.

Resection of subvalvar left ventricular outflow tract obstruction was performed in 11 patients, including 2 of the 4 who underwent a Ross procedure. Subvalvar membranectomy and myectomy were employed as indicated in each of these patients.

Two patients underwent procedures on the coronary arteries. One patient, who had diffuse left main coronary artery stenosis, underwent coronary artery bypass grafting with anastomosis of a pedicled left internal mammary artery graft to the left main coronary artery. Another patient underwent endarterectomy of the left coronary ostium. A third patient underwent separation of the right coronary leaflet from the supravalvar ridge to which it was completely fused, thus reestablishing continuity between the aorta and the previously isolated right coronary system.

Other procedures
Seven of the 12 patients with Williams syndrome underwent augmentation of the pulmonary arteries with a patch. In 6 of these patients, augmentation consisted of an inverted-Y patch extending into the sinuses of the pulmonary root, along with extensive augmentation of the branch pulmonary arteries and partial pulmonary valvectomy.


   Results
Top
 Abstract
 Introduction
 Patients and methods
 Results
Comment
 References
 
Early results
There was 1 perioperative death, which occurred 2 days after repair in a 2-year-old patient with Williams syndrome who underwent augmentation of the supravalvar aortic region and the central and branch pulmonary arteries. This patient developed progressive acidosis despite increasing inotropic support, and went into cardiac arrest from which she could not be resuscitated. There were no other deaths, significant complications, or reoperations in the perioperative period. The median duration of cardiopulmonary bypass was 137 minutes (49 to 346 minutes), and the median duration of cardioplegic arrest was 59 minutes (9 to 145 minutes). Patients remained in the hospital for a median of 6 days after surgery (2 to 30 days).

Late results
At a median follow-up of 33 months (2 to 74 months), all 35 patients who survived to hospital discharge were alive. One patient underwent a Ross procedure 1 month after repair of supravalvar stenosis and aortic valvuloplasty. This patient had moderate aortic regurgitation before repair of the supravalvar obstruction, but because the obstruction was primarily supravalvar and extensive valvuloplasty and leaflet thinning were performed on the dysplastic trileaflet aortic valve, an attempt was made initially to repair the supravalvar stenosis without replacing the valve. The patient returned with generalized malaise and persistent fever, and was found to have continued moderate regurgitation despite the valvuloplasty. Two other patients had undergone replacement of the aortic valve with a mechanical prosthesis 3 and 5 years after repair of the supravalvar stenosis. Both of these patients had multilevel obstruction and developed severe aortic regurgitation after repair of supravalvar stenosis with an inverted-Y patch (n = 1) and a Ross procedure (n = 1). At the time of aortic valve replacement, both underwent augmentation of the ascending aorta, and 1 underwent a Konno procedure and enlargement of the right coronary ostium, which had not been stenotic at the time of the original repair. Among surviving patients, the median pressure gradient across the left ventricular outflow tract was 10 mm Hg (0 to 35 mm Hg). A residual gradient greater than 25 mm Hg was present in 5 patients, 4 of whom had preoperative gradients of 90 mm Hg or more (3 above 100 mm Hg). Aside from the patients who required reoperation, significant aortic regurgitation had not developed in any patient.


   Comment
Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
References
 
The anomaly commonly referred to as "supravalvar aortic stenosis" is often a condition not simply of supravalvar pathology, but of aortic valvar and root involvement as well [1]. This is a critical differentiation, for proper function of the aortic valve depends upon the integrity of the entire root [10, 11]. Accordingly, the optimal surgical management of this anomaly should strive for preservation and restoration of the aortic valve and root, rather than simply for relief of the supravalvar stenosis. Surgeons have demonstrated an increasing appreciation for this concept, as initial techniques of repair utilizing a diamond-shaped or oval patch extending across the stenosis into a single sinus of Valsalva were refined by extending augmentation patches into 2 or all 3 sinuses [2, 6], with subsequent progression to excision of the abnormal aortic wall tissue at the sinotubular junction and down into the sinuses [7]. Although the optimal means of relieving supravalvar obstruction while preserving the integrity of the aortic root has remained a central issue in the literature on this topic, little attention has been devoted to the question of how best to manage abnormalities of other elements of the aortic root and adjacent structures in these patients, such as the aortic valve, subvalvar region, and coronary arteries.

Abnormalities of the aortic root and adjacent structures are common in patients with supravalvar stenosis (Table 1). In surgical series, significant involvement of the aortic valve, including stenosis or an abnormal number of leaflets, has been reported in 34% to 47% of patients, and was present in 24% of cases reviewed by Petersen and coworkers [13]. Our experience has been similar, with a bileaflet valve or valvar stenosis in 18 of 36 patients. Although a bileaflet valve is common, it is likely that abnormal stresses on and restriction of the leaflets in the setting of impaired distensibility of the sinotubular junction lead to degenerative changes as well [10, 14]. In previous series, obstruction of the subvalvar left ventricular outflow tract has been found in 13% to 20% of patients, and significant aortic regurgitation reported in up to 23% (Table 1). Each was present in 31% of our patients.


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  		Table 1. Associated Pathology of the Left Ventricular Outflow Tract in Published Series of Repair of Supravalvar Aortic Stenosis

 
In previous surgical series, procedures on the aortic valve have been performed in up to 42% of patients undergoing repair of supravalvar aortic stenosis (Table 2). In most cases, simple commissurotomy was performed for partial fusion of a bicuspid valve. One-third to half of the procedures performed on the aortic valve in these series, however, have been valvar replacement with a mechanical or bioprosthesis. In the present era, the use of prostheses for replacement of the aortic valve in children is contentious, insofar as the Ross procedure has provided another, arguably superior, option in many patients requiring replacement [15]. In addition, congenital heart surgeons today have become more aggressive about salvaging the native valve with valvuloplasty, whether it be augmentation, suspension, commissuroplasty, annuloplasty, or thinning. The role of the Ross procedure in the management of supravalvar aortic stenosis with associated valvar, with or without subvalvar, pathology has not been elucidated. Over the past several years, this procedure has become an important strategy in the management of systemic outflow tract disease in children. In addition to older patients with combined aortic regurgitation and stenosis, replacement of the aortic valve with the pulmonary autograft has revolutionized the surgical management of complex, multilevel obstruction of the left ventricular outflow tract in infants and young children. In the sole report of which we are aware that addresses the issue of how to manage the aortic valve in patients with supervening supravalvar stenosis, the authors conclude that a Ross procedure at the time of repair of supravalvar stenosis may be the preferred option in patients with a bileaflet valve [8]. Seven of 19 patients with supravalvar stenosis had a bileaflet valve and did not undergo repair or replacement at the time the supravalvar stenosis was repaired. Of these 7, 5 eventually required reoperation on the aortic valve. We have performed a Ross procedure in 4 patients with supravalvar stenosis, and all have done well except for 1 who required replacement. An additional patient underwent a Ross procedure 1 month after repair of supravalvar stenosis, and probably should have had the aortic valve replaced at that time. All 5 of these patients had a bileaflet aortic valve, which is consistent with the conclusions of Delius and associates [8].


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  		Table 2. Associated Surgical Procedures on the Left Ventricular Outflow Tract in Published Series of Repair of Supravalvar Aortic Stenosis

 
Despite the frequency of procedures on the aortic valve in previous series, perhaps the major shortcoming of the earlier experience with surgical treatment of this lesion has been inadequate management of the aortic valve. In one series, an abnormal aortic valve was the only independent predictor of late mortality [5]. The most frequent indication for reoperation after repair of supravalvar aortic stenosis has been aortic valvar dysfunction [3–5]. Reoperations for left ventricular outflow tract obstruction or aortic regurgitation have been required in 17%–40% (nonactuarial) of early operative survivors [3–5]. Notably, in 2 large studies, abnormalities of the aortic valve [5] or simply a bileaflet aortic valve [4] at the time of the original repair of supravalvar stenosis were the sole risk factors for late reintervention. Although the specific indication for late repair or replacement of the aortic valve in these series generally has not been stated, both stenosis and regurgitation are likely encountered. Regurgitation may be due to distortion of the aortic root during augmentation of the ascending aorta, abnormal root mechanics due to anisotropy of the augmented sinuses and sinotubular junction, progressive immobility of the valve as a result of thickening or insufficient mobilization at the original procedure, prolapse, or infective endocarditis. Stenosis may result from progression of valvar thickening/calcification in the face of inadequate relief of valvar obstruction at the original procedure, disease of a native bicuspid valve, or outgrowth of a prosthetic valve. The fact that 3 of our patients have required aortic valve replacement highlights the fact that the aortic valve is likely to be the primary source of problems after repair of supravalvar obstruction in these patients, even with careful attention to the management of the aortic valve. All 3 of these patients had complex left ventricular outflow tract disease, which likely places them at risk for a more complicated evolution of disease, and underscores the importance of proper management of the entire systemic outflow tract in patients with supravalvar aortic stenosis.

Fusion of the edges of the aortic valvar leaflets to the supravalvar ridge is a well-recognized feature of supravalvar aortic stenosis that occurs with a spectrum of severity. In addition to impairing valvar function, this process can affect coronary arterial flow as well, with implications that are commensurate with the extent of fusion and the number of leaflets involved. Although partial fusion of the left or right coronary leaflets of the aortic valve to the supravalvar ridge is not uncommon [16, 17], complete fusion and consequent isolation of a coronary artery from the aortic lumen has been recognized in only a few cases prior to the patient described in the present report [13, 18, 19]. In addition to the 4 reported cases (including ours) of this abnormality in patients with supravalvar aortic stenosis, at least 3 cases have been published of coronary artery isolation resulting from fusion of a valvar leaflet to the aortic wall in the absence of other anomalies [20–22]. In the great majority of these cases, it was the left coronary artery that was excluded or atretic. Fusion of the leaflet to the aortic wall has also been reported after patch augmentation of the ascending aorta for repair of supravalvar aortic stenosis [7]. This lesion may produce typical signs and symptoms of ischemia, but by virtue of its progressive development, adequate recruitment of collateral flow from the contralateral coronary system appears to occur, thus preventing outright infarction. In addition to isolation of a coronary arterial orifice, the coronary arteries may be of concern in patients with supravalvar aortic stenosis for several reasons. As was the case in 2 of our patients and several previously reported cases, stenosis [5] or atresia [23–25] of a coronary ostium and proximal coronary artery may occur, necessitating either ostial endarterectomy, coronary bypass grafting, or other reconstructive techniques [25]. Thus, as Martin and coworkers suggest, it is important to inspect the coronary ostia in all patients undergoing repair of supravalvar aortic stenosis [25]. In addition, because the coronary arteries lie proximal to the site of supravalvar obstruction, they are exposed to supranormal pressures and may suffer as a result. As several authors have noted [6, 13, 14], dilatation of the coronary arterial system may occur in patients with this lesion, and may be associated with medial thickening and initimal fibrosis. Although the long-term significance of this process has not been confirmed clinically, it is intuitive to suppose that such patients may be at increased risk of developing atherosclerotic coronary artery disease. It is clear that subendocardial ischemia can ensue in the presence of supravalvar stenosis [26], though the coronary arterial hypertension that occurs may mitigate the mismatch of perfusion pressure and mural pressure that is present in severe obstruction of the left ventricular outflow tract proximal to the coronary arteries. Although the time frame of coronary pathogenesis in the face of elevated pressures resulting from supravalvar obstruction is not known, it has been documented that coronary dilatation decreases early after repair of supravalvar obstruction, and systolic distention of the coronary arteries (and aortic root) is decreased as well [14].

Data on the natural history of supravalvar aortic stenosis are limited. The few studies that have been published suggest that supravalvar aortic stenosis is a progressive disease, in the sense that the pressure gradient across the stenotic region increases with time [27, 28]. To the best of our knowledge, there is no way yet to predict which patients will develop significant progression of disease. In the context of a disease that begets potentially significant secondary changes in the aortic valve, coronary arteries, and left ventricular myocardium, the inability to predict progression begs the question of when repair should be undertaken, in terms of both age and stage of disease. The impact of age and severity of supravalvar obstruction at the time of repair are not clear. In theory, younger age at repair may be associated with more severe disease and increased probability of recurrence, insofar as growth is likely to play a more significant role in such patients. More severe obstruction at the time of repair may also be associated with less favorable outcome, both from the perspective of residual obstruction (80% of patients with residual gradients > 25 mm Hg in our series had preoperative gradients of 90 mm Hg or more) and secondary coronary arterial and left ventricular damage. As with all surgical decisions, the answer to this question must be reached through risk-benefit analysis. The potential benefits of effective repair as early in the course of disease as possible are substantial. In contrast, surgical mortality and morbidity are uncommon with this disease, regardless of age at repair. For example, we have had only 1 perioperative death in our experience, along with a single early reoperation. The risks of surgery are likely to be relatively lower in patients with less severe compromise and better functional reserve, and it is no longer the case that young age, in and of itself, places patients at increased risk for poor outcome.

Risk factors for recurrence of supravalvar aortic stenosis are not well-known. In long-term follow-up studies, there is no strong evidence to suggest that this is a commonly recurrent disease [3–5]. Similarly, in our experience, residual supravalvar obstruction has been stable in all patients, with no evidence of progression. All 5 patients with residual outflow tract gradients greater than 25 mm Hg had this degree of obstruction immediately after surgery. However, as discussed earlier, valvar disease constitutes an important cause of late morbidity. At the present time, it seems reasonable to suggest repair of supravalvar stenosis and associated lesions at the time of presentation or when the gradient across the left ventricular outflow tract reaches a moderate degree of severity. The median gradient across the left ventricular outflow tract in this series was 70 mm Hg, which is markedly lower than in previously published series, in which ~90 mm Hg was average [3–5]. Similarly, the median age at repair of 4 years was lower than most series [3–5]. Whether earlier repair will prove to be beneficial in ameliorating the secondary pathologic processes that can result in the setting of supravalvar aortic stenosis remains to be seen. Additional long-term follow-up, as well as studies of myocardial function in patients with this disease, will be necessary to substantiate the rationale of early repair.


   Footnotes
 
This article has been selected for the open discussion forum on the STS Web site: http://www.sts.org/section/atsdiscussion/


   References
Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 

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Accepted for publication July 8, 1999.




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