Late Outcomes for Surgical Repair of Supravalvar Aortic Stenosis

Background. We reviewed our experience with the surgical management of supravalvar aortic stenosis (SVAS) to determine long-term outcomes and factors related to late reoperation. Methods. Between August 1956 and May 2009, 78 patients (50 males) underwent surgical correction of SVAS. Median age was 10.4 years (range, 16 days to 55.2 years). Mean preoperative gradient was 57.2 +/- 21.9 mm Hg with a mean peak gradient of 99.5 +/- 34.8 mm Hg. Supravalvar aortic stenosis was discrete in 51 patients (64%) and diffuse in 27 patients (35%). Aortic valve stenosis was present in 22 patients (29%). Williams-Beuren syndrome was present in 32 patients (41%). Results. Surgery was either a diamond-shaped patch in 67 patients (85.9%) or a pantaloons-shaped patch in 11 patients (14.1%). Aortic valve intervention was required in 20 patients (25.64%). Mean gradient immediately after repair was 25 +/- 25 mm Hg, with 13 patients (16.7%) having a residual gradient. A high residual gradient was more likely in the diffuse group (odds ratio, 3.73; 95% confidence interval, 1.07 to 12.98). There were 2 (2.6%) early deaths, both with diffuse SVAS. Median follow-up was 19.8 years; maximum was 48.5 years. The mean gradient across the left ventricular outflow tract at late follow-up was 8.8 mm Hg (95% confidence interval, 3.7 to 14.01). Overall survival was estimated at 90% +/- 7%, 84% +/- 9%, and 8% 2 +/- 10% at 5, 10, and 20 years, respectively. Predictors of mortality were age younger than 2 years (p = 0.021), diffuse SVAS (p = 0.045), aortic valve stenosis (p = 0.032), and high postoperative gradient (p = 0.023). Presence of Williams-Beuren syndrome did not affect survival (p = 0.305). Freedom from late reoperation was 97% +/- 4%, 93% +/- 7%, and 86% +/- 10% at 5, 10, and 20 years, respectively. Significant aortic valve disease (p < 0.001) and diffuse SVAS (p = 0.009) were risk factors for late reoperation. Conclusions. Surgical repair for SVAS can be performed with a single-patch technique with good long-term outcome. Late mortality and need for reoperation are more likely with diffuse SVAS or the presence of aortic valve stenosis.