Surgical repair of congenital supravalvular aortic stenosis in children.

Abstract

Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly characterized by varying degrees of left ventricular outflow tract obstruction beginning distal to the aortic valve. Between March 1962 and December 2000, 101 consecutive patients underwent surgical correction for congenital SVAS at Riley Children's Hospital. There were 61 male (60%) and 40 female (40%) ranging in age from 3 month to 17 years (medium age, 6.1 years). Fourteen patients (14%) had Williams syndrome. Preoperatively, 11 patients were in New York Heart Association (NYHA) functional class I, 55 in class II, 28 in class III, and seven in class IV. Of the 101 patients, 73 (72%) had localized type SVAS and 28 (28%) diffuse type SVAS. Those with localized SVAS were successfully treated with patch aortoplasty, whereas those with diffuse SVAS required either an apical aortic conduit or extensive endarterectomy with patch aortoplasty. The overall mean pressure gradient was reduced to 21 mmHg (P<0.001) in the early postoperative period. There were one early death (<30 days postoperatively) (1%), two (2%) late deaths, and 14 patients (14%) underwent one or two additional operation (n=17) in a follow-up period ranging from 6 months to 30 years (medium 9.4 years). Postoperatively, there were 72 patients (73%) in NYHA functional class I and 26 (27%) in class II. Overall survival including operative mortality was 98% at 10 years, 97% at 20 and at 30 years. Good surgical outcome of congenital SVAS can be achieved with the appropriate method of treatment in patients with both localized and diffuse SVAS.