[Congenital aortic supravalvular stenosis. Frequency and results of surgical treatment in a third level hospital].

In a prospective study made between 1 September 1996 and 31 December 2001, pediatric patients with a diagnosis of supravalvular aortic stenosis confirmed by a reduction in the aortic inner diameter and a gradient > or = 50 mmHg were detected. Of 83 patients with aortic stenosis, only 7 (8.4%) had supravalvular aortic stenosis. All 7 patients underwent surgical treatment consisting of resection of fibrous tissue and reconstruction of the ascending aorta with a preclotted Dacron patch. One patient with severe, diffuse stenosis died and the another had perioperative heart failure, cardiac arrest and reversible neurological sequelae. A significant decrease in the postoperative gradient was obtained (p < 0.05). At present all surviving patients are free of symptoms. It was concluded that supravalvular aortic stenosis is infrequent in Mexico. In our experience, surgical treatment produced good results and success depended on the magnitude and type of stenosis.