SUPRAVALVAR AORTIC-STENOSIS - A 29-YEAR REVIEW OF SURGICAL EXPERIENCE

Between February 1960 and August 1989, 73 consecutive patients underwent surgical correction for supravalvar aortic stenosis (SVAS) at the Texas Heart Institute. There were 43 male (59%) and 30 female patients (41%) ranging in age from 5 days to 27 years (mean age, 12 years). Preoperatively, 8 patients were in New York Heart Association functional class I, 43 in class II, 18 in class III, and 4 in class IV. Of the 73 patients, 62 had localized SVAS and 11 (15%), diffuse SVAS. For all procedures, patients were placed on cardiopulmonary bypass. Those with localized SVAS were successfully treated with patch aortoplasty, whereas those with diffuse SVAS required either an apicoaortic conduit or extensive end-arterectomy with extended patch aortoplasty. There were eight early deaths (less-than-or-equal-to 30 days postoperatively) (11%) and four late deaths (> 30 days postoperatively) (6%) in a follow-up period ranging from 2 months to 28 years. Sixteen patients (25%) underwent one or more additional operations in the follow-up period. Postoperatively, there were 44 patients in New York Heart Association functional class I and 17 in class II. Preoperative functional class III and class IV (p < 0.0005), diffuse SVAS (p = 0.05), and the presence of associated congenital defects (p < 0.01) were important determinants of death.