Surgical Repair of Supravalvar Aortic Stenosis in Association With Transverse and Proximal Descending Aortic Abnormalities

Abstract

Background

Supravalvar aortic stenosis (SVAS) may be an isolated defect of the proximal ascending aorta. However, more severe cases have extension of the arteriopathy into the transverse and proximal descending aorta. The purpose of this study was to review our experience with SVAS with and without aortic arch arteriopathy.

Methods

This was a retrospective review of 58 patients who underwent surgical repair of SVAS. The median age at repair was 18 months. A total of 37 patients had Williams syndrome. A total of 31 (53%) patients had associated peripheral pulmonary artery stenosis and 23 (39%) had coronary artery ostial stenosis (CAOS).

Results

A total of 37 of 58 (64%) patients had surgical repair of SVAS without the need for arch intervention while 21 (36%) patients had repair of the distal aortic arch. There were 3 (5.2%) operative deaths, 2 of whom had aortic arch involvement and one without arch involvement. There were 2 deaths after discharge from the hospital. Patients who needed arch surgery were more likely to have severe arch gradients compared to those without arch involvement (71% vs 30%, P < .05), were more likely to undergo concomitant procedures for peripheral pulmonary artery stenosis or CAOS (90% vs 62%, P < .05), and to have Williams syndrome (86% vs 51%, P < .05).

Conclusions

More than one-third of patients who had SVAS repair at our institution had procedures directed at the transverse or proximal descending aorta. Patients with arch involvement had more severe arch obstruction, required more concomitant procedures, and were more likely to have Williams syndrome.

Keywords

outcomes congenital heart surgery congenital heart disease

Introduction

Supravalvar aortic stenosis (SVAS) is a relatively uncommon form of congenital heart defect accounting for less than 1% of all surgical cases in the Society for Thoracic Surgeons Congenital Cardiac Surgery Database.¹ SVAS is characterized anatomically by a narrowing of the proximal-most portion of the ascending aorta, with the narrowest portion often found at the level of the sino-tubular junction.² The aorta above the sino-tubular junction often widens out to attain a normal caliber well before the origin of the innominate artery. SVAS can also be characterized physiologically based on the gradient across the narrowed portion of the aorta, with mild considered as gradients less than 20 mmHg, moderate as gradients of 20 to 50 mmHg, and severe as gradients of 50 mmHg or greater.^3,4

SVAS is often associated with Williams syndrome (7q11.23 microdeletion) and Elastin arteriopathy.^5,6 Both of these syndromes have a genetically mediated arteriopathy that manifests as a markedly thickened (3-5 X normal) aortic wall due to hypertrophy of the aortic media.⁷ This arteriopathy may extend into the transverse aortic arch and proximal descending aorta, adding an additional physiologic burden to the heart. The thickened proximal aortic wall may also encroach on the coronary artery ostia resulting in coronary artery ostial stenosis (CAOS).^8,9 The combination of moderate or severe SVAS in combination with CAOS can create an unstable balance of myocardial oxygen supply and demand that may predispose to sudden cardiac arrest.^10,11

Many patients with Williams syndrome and Elastin arteriopathy will also have peripheral pulmonary artery stenosis.^12–15 The pulmonary arteriopathy associated with these 2 syndromes almost always is characterized by ostial disease located at the bifurcation of the lobar and segmental branches.^16–19 The surgical treatment of peripheral pulmonary artery stenosis has been shown to be effective in reducing pulmonary artery and right ventricular pressures.^20,21

Numerous surgical techniques have now been described for repair of SVAS.^22–30 The aim of these techniques is to enlarge the proximal ascending aorta and thus relieve the physiologic obstruction of blood flow. By doing so, the excess pressure-load to the heart is reduced (or eliminated), and the balance of myocardial oxygen supply and demand moved to a more stable and sustainable situation. The 2-patch and 3-patch techniques have a further goal of restoring a more normal geometric left ventricular outflow tract and presumably, this will result in preserving aortic valve function.^31,32

Despite the fact that there are many papers going back for more than 40 years on the subject of the surgical repair of SVAS, little attention has been paid to the implications of arteriopathy extending into the aortic arch. The purpose of this study was to review our surgical experience with SVAS repair with and without aortic arch involvement.

Materials and Methods

This was a retrospective review of 58 patients who underwent surgical repair of SVAS at our institution from 2004 to 2021. The study was approved by the Institutional Review Board (IRB) at Stanford University (Protocol ID 48389 approved on 10/16/2018 and Protocol ID 42875 approved on 8/24/2018).

Our surgical approach to patients who have SVAS and aortic arch narrowing is direct cannulation of the innominate artery.³³ The patients are cooled to 25°C and antegrade cerebral perfusion is implemented by clamping the proximal innominate artery, left carotid artery, and left subclavian artery. Flow is then reduced to 40 mL/kg/min and the aortic arch opened with flow return from the descending thoracic aorta collected via cardiotomy suckers. The aorta is then augmented with a patch of pulmonary artery homograft. Once the homograft patch has been sutured to a position beneath the innominate artery, a cross-clamp can be placed at that level and the clamps on the great vessels removed. Full flow is then resumed at 100 mL/kg/min. Using this technique, the time period of antegrade cerebral perfusion is generally less than 30 min.

Results are reported as means ± standard errors, and medians and ranges where appropriate. Comparison of groups was performed using Chi-square analysis. Kaplan-Meier survival analysis was performed for both survival and freedom from re-operation.

Results

The median age at the time of repair was 18 months (range 1.5 months to 19 years). The age of the patients at the time of surgery is shown in Figure 1. These data are color coded in the histogram to depict those patients who underwent repair of the transverse and/or proximal descending aorta versus those who did not have a repair of the distal arch. The median age of patients with arch involvement was 18 months, and the median age of patients without arch involvement was 22 months (not significantly different).

Forty patients were undergoing their first cardiac surgical procedure while 18 were undergoing a re-operation. For the 18 patients undergoing re-operation, 12 had undergone a previous procedure on the left ventricular outflow tract, 10 had undergone a previous procedure on the right ventricular outflow tract, with 4 had undergone previous procedures on both the left and right ventricular outflow tracts.

Thirty-seven patients had Williams syndrome confirmed by genetic testing. Other diagnoses include Elastin Arteriopathy (n = 7), Shone's syndrome (n = 4), complex aortic and subaortic valve disease (n = 4), chromosome 19 mutation (n = 1), and no associated syndrome or predisposing anatomy (n = 5).

Fifty-four patients underwent a preoperative cardiac catheterization, 18 patients had a preoperative computed tomography (CT) scan, and 4 patients had a magnetic resonance imaging (MRI) study.

Twenty-six patients had severe SVAS, 24 had moderate SVAS, and 8 had mild SVAS. Thirty-one patients (53%) had moderate or severe peripheral pulmonary artery stenosis, and this was the primary indication for surgical intervention in all 8 patients with mild SVAS and in 16 of the 24 patients with moderate SVAS. In addition, 23 patients (39%) had the presence of CAOS known preoperatively based on a history of cardiac arrest (n = 7) or studies documenting coronary stenosis (n = 16). Four additional patients had the diagnosis of CAOS made intraoperatively based on direct inspection of the ostia. CAOS was the primary indication for surgical intervention in 10 patients. A Venn diagram demonstrating the presence of moderate or severe SVAS, the presence of moderate or severe peripheral pulmonary artery stenosis, and the presence of CAOS is shown in Figure 2.

Repair of the SVAS was performed using a single-patch technique in 12, a 2-patch technique in 22, and a 3-patch technique in 24. The median cross-clamp time was 48 min (range 22–135 min) and the median cardiopulmonary bypass time was 205 min (range 55–473 min).

Twenty-one of the 58 patients (36%) had surgical repair of SVAS and repair of the transverse or proximal descending aorta. Patients with arch involvement were more likely to have obstruction graded preoperatively as severe compared to patients without arch involvement (71% vs 30%, P < .05). These data are shown in Figure 3.

Eighteen of the 21 (86%) patients with arch involvement had Williams syndrome compared with 19 of 37 (51%) without arch involvement (P < .05). These data are shown in Figure 4.

Nineteen of the 21 patients (90%) with arch involvement underwent concomitant procedures for repair of CAOS or peripheral pulmonary artery stenosis (6 had both) compared with 23 of 37 (62%) patients who underwent concomitant procedures (10 had both) without arch involvement (P < .05). These data are shown in Figure 5.

Thirteen of the 21 (62%) patients with arch involvement had peripheral pulmonary artery stenosis compared with 18 of 37 (49%) without arch involvement (Chi-square statistic = 0.9461, P value = .33). The mean preoperative right ventricle to aortic pressure ratio was 0.98 ± 0.12 and decreased to a mean of 0.35 ± 0.07 postoperatively. Twelve of the 21 (57%) patients with arch involvement had CAOS compared with 15 of 37 (41%) without arch involvement (Chi-square statistic = 1.48, P value = .22).

There were 3 operative deaths (5.2%), all of whom had Williams syndrome and all of whom required concomitant procedures to address hemodynamically significant lesions. Two of the 3 operative deaths occurred in patients who underwent distal arch repair, while one operative death occurred in a patient who did not undergo arch repair. Both patients with arch involvement also had CAOS. One of these 2 patients was 6-weeks-old and sustained an ischemic myocardial injury intraoperatively, required extracorporeal membrane oxygenation (ECMO) support postoperatively, but never recovered ventricular function. The second patient with arch involvement and CAOS was 17-months-old and developed profound low cardiac output postoperatively, was placed on ECMO, and also did not survive. The patient who did not have arch involvement did not have CAOS but did have peripheral pulmonary artery stenosis. This patient underwent repair of the ascending aorta and peripheral pulmonary artery stenosis but sustained a cardiac arrest in the intensive care unit and did not survive.

Median duration of follow-up was 4.1 years (range 3 months to 15 years). There have been 2 deaths following discharge from the hospital. Both of these patients did not have distal arch involvement. One of these 2 patients had CAOS and underwent concomitant repair along with the SVAS repair. This patient sustained a cardiac arrest 2 months postoperatively, presumably from an ischemic cause. The other patient did not have distal aortic arch involvement and died 4.4 years after SVAS repair following an acute respiratory illness. The actuarial survival for the entire cohort is shown in Figure 6A with a 5-year estimated survival of 89%. The actuarial survival curve for those with and without arch involvement is shown in Figure 6B, with a 5-year estimated survival of 90% for patients with arch involvement and 88% for those without arch involvement.

Nine patients (ie, 16.3% of the survivors) have undergone re-operations following their SVAS repair. This includes 7 patients who had re-operations on the left side of the circulation. Two of these patients initially had mild arch obstruction but developed increased gradients following SVAS repair. A third patient had undergone a whole arch repair but subsequently developed narrowing of the descending thoracic aorta down to the level of the diaphragm. Another patient required revision of a previously augmented transverse and distal aortic arch. No patients have undergone catheter-based interventions on the arch/descending aorta following aortic arch surgery.

Three other patients required re-operations to address a dysfunctional aortic valve including one patient who underwent a Ross-Konno procedure and 2 patients who had an aortic valve replacement (all 3 of these patients had Shone's syndrome). Three patients have undergone re-operations on the right side of the circulation to address peripheral pulmonary artery stenosis. Two of these patients did not undergo an initial repair due to the presence of CAOS and a history of cardiac arrest. The third patient had separate re-operations on both the right and left sides of the circulation. The Kaplan-Meier freedom from re-operation for the cohort of 55 survivors is shown in Figure 7A. The estimated freedom from re-operation at 5 years was 86%. The Kaplan-Meier freedom from re-operation for the subgroups of those with and without arch involvement is shown in Figure 7B.

Discussion

This study was performed to evaluate the surgical results of SVAS repair at our institution. Thirty-eight percent of the patients in this series underwent SVAS repair in conjunction with distal aortic arch surgery. In addition, 53% of the patients had peripheral pulmonary artery stenosis and 39% had CAOS. Patients who underwent aortic arch repair were more likely to have severe SVAS, more likely to undergo concomitant procedures, and more likely to have Williams syndrome. These results suggest that SVAS is often associated with other significant hemodynamic lesions and belies the predominant sentiment in the literature that SVAS is an isolated lesion.

Our approach to addressing SVAS is tailored to the anatomy and physiology of the given patient. In general, we use a single-patch technique for mild SVAS, a two-patch technique for moderate SVAS, and a three-patch technique for severe SVAS or those who require aortic arch augmentation.

Twenty-one patients (or 38% of the entire cohort) in this series had SVAS in association with aortic arch narrowing. All of these patients underwent a three-patch technique facilitated by the use of innominate artery cannulation and regional perfusion. In most cases, the severity of the arteriopathy gradually lessens as one continues more distally on the transverse arch and proximal descending aorta. There are some ideal patients in whom the distal arch or proximal descending aortic tissue will completely normalize allowing the patch to extend to nondiseased tissue. Other patients will have a continuation of mild or moderate arteriopathy of the proximal descending aorta in which case a decision must be made as to how far to carry the incision and patch. It should also be noted that there were 3 patients who had progression of their arteriopathy during the postoperative time-period of observation and required re-operation to address these areas that had progressed.

There were a total of 27 patients (47% of the entire cohort) who had surgical repair of CAOS. This included 23 patients in whom the diagnosis was known preoperatively based on a history of cardiac arrest or on preoperative imaging studies and 4 patients in whom the diagnosis was made intraoperatively. We have recently described our surgical approach to CAOS in patients with Williams and Elastin arteriopathy syndromes.⁹ This approach entails an incision in the aorta that continues through the ostia and onto the left main or right coronary artery. The incision is then patch augmented using pulmonary artery homograft. We favor this surgical approach, as it also effectively splits the overhanging shelf created by the SVAS.³⁴ We have been quite satisfied with this surgical approach, but because the numbers are small, it is not possible to comment on whether this approach is superior to that of excising tissue at the coronary ostia as described by Luo et al.³⁵

Thirty-one of the 58 patients (53%) in this study had moderate or severe peripheral pulmonary artery stenosis. This prevalence of peripheral pulmonary artery stenosis is not all that surprising given that 44 patients (76%) had either Williams syndrome or Elastin arteriopathy. These 2 syndromes are frequently associated with peripheral pulmonary artery stenosis, whereas most other syndromes associated with SVAS do not have an association with peripheral pulmonary artery stenosis. Thus, the more heavily weighted a series is towards Williams and Elastin arteriopathy, the more one can expect coexistent peripheral pulmonary artery stenosis.

Eight patients in this series had severe peripheral pulmonary artery stenosis in association with mild SVAS. In these 8 patients, the indication for surgical intervention was the presence of systemic right ventricular pressures. Given that the patients were already undergoing surgery utilizing cardiopulmonary bypass, the decision was made in each case to address the mild SVAS. However, it is recognized that these patients would not have met criteria for an isolated SVAS repair. If one were to exclude these 8 patients with mild SVAS, it would adjust the numerator and denominator of several of our prevalence calculations. For example, none of the 8 patients with mild SVAS had distal arch narrowing, and therefore if these patients were excluded the prevalence of distal arch abnormalities would increase from 36% to 42%. Similarly, none of the 8 patients with mild SVAS had CAOS, and therefore the prevalence of CAOS would increase from 39% to 46%. The prevalence of Williams syndrome would also increase in the series from 64% to 70%. Conversely, since all 8 of these patients did have peripheral pulmonary artery stenosis, the prevalence of this entity would decrease from 53% to 46% of these patients were excluded.

There were 3 early and 2 late deaths in this series, with a predicted 5-year survival of 89% (Figure 6A). The data indicate that patients with and without aortic arch abnormalities had similar survival (Figure 6B). It is reassuring that the mortality data was similar for these 2 subgroups despite the fact that patients with arch involvement had higher gradients and underwent more concomitant procedures. Two of the 3 operative deaths had very high aortic gradients (90 and 100 mmHg) in combination with severe left main CAOS. The third operative death occurred in a patient who did not have a distal aortic arch disease but did have severe peripheral pulmonary artery stenosis. One of the late deaths also occurred in a patient who had severe left main CAOS. Thus, 4 of the 5 deaths in this series occurred in patients with a significant hemodynamic lesion separate from their diagnosis of SVAS.

One of the challenges in managing patients with SVAS in the presence of CAOS and/or peripheral pulmonary artery stenosis is deciding the priority of what will and what will not be addressed in any given surgery. While we have been an advocate for repairing all hemodynamic burdens in one operation, this can also result in very prolonged periods of cardiopulmonary bypass. In a study of 100 patients undergoing complex pulmonary artery reconstructions with cardiopulmonary bypass times in excess of 300 min, the median number of complications was 4, and median hospital length of stay was 24 days.³⁶ Thus, this may be the “price” that one must pay for performing procedures with very long cardiopulmonary bypass times (there was a sole mortality resulting in a 99% rate of survival to discharge). In the current study, there were 2 patients with a history of sudden cardiac arrest in whom the decision was made preoperatively not to address the presence of concomitant peripheral pulmonary artery stenosis. As a matter of policy, we would always prioritize the repair of significant SVAS gradients and CAOS above all other considerations.

The current literature on the subject of surgical repair of SVAS has a paucity of information regarding the prevalence of distal arch obstruction or its potential impact. In the largest series reported to date on the surgical treatment of SVAS, the European Congenital Heart Surgeons Association published a multicentric study that included 301 patients.²² In this study, 25 of 301 patients (8%) were reported to have the diagnosis of aortic arch hypoplasia and/or coarctation and only 4% underwent concomitant aortic arch repair. In a recent single-center series from Texas Children's Hospital, the prevalence of concomitant aortic arch surgery was 9%,³¹ and in an older historical series from Boston Children's, it was 6.7%.³⁷ It is difficult to account for the 4-fold difference in aortic arch abnormalities found in the current study compared with previous studies, particularly since the prevalence of Williams syndrome was similar (58%, 45%, and 61% in these 3 studies compared with 64% in our study). It is conceivable that a willingness to treat a complex condition such as distal arch obstruction results in an increase in the recognition and diagnosis of that condition within the confines of an institution.

In summary, in this study, we found that more than one-third of patients with SVAS had aortic arch involvement. This prevalence of distal arch involvement is considerably higher than is currently reflected in the literature. Our data would suggest that aortic arch involvement is associated with a more severe form of disease that is closely linked to the presence of Williams syndrome. We would conclude that the diagnosis of SVAS is not nearly as uniform as previously described and in our opinion should be bifurcated into a simpler and more aggressive form.

Footnotes

Declaration of Conflicting Interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

ORCID iD

Richard D. Mainwaring

Abbreviations and Acronyms

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