Results of surgical repair of congenital supravalvular aortic stenosis.

Our experience with congenital supravalvular aortic stenosis dates from 1977 to 1991. Thirteen patients, aged 2 days to 38 years (mean 7.6 years, median 3.8 years), had surgical repair of the lesion. The preoperative peak-to-peak systolic gradients ranged from 25 to 110 mm Hg (mean 64 mm Hg). Four patients had trace to mild aortic insufficiency. Surgical repair was accomplished by several techniques that have evolved over time. There was one death in a 2-day-old neonate who also had severe hypertrophic cardiomyopathy. The 12 survivors had postoperative gradients of 0 to 30 mm Hg (mean 10 mm Hg) in the supravalvular region. The mean reduction in gradient was 48 mm Hg. A new technique employing all autologous aortic tissue is described.