Supravalvular aortic stenosis is a rare form of left ventricular outflow tract obstruction and it can be in local or diffuse form. The disease may be inherited as an auto somal dominant trait, although sporadic cases also ocur. Except the aorta, peripheral pulmonary arteries and the other major arteries can be involed.

At Koşuyolu Heart and Research Hospital, 8 patients underwent surgically correction with the diagnosis “supravalvular aortic stenosis” since 1985. 5 of them (62.5%) were male and 3 (37.5%) of them were female, with mean age 14.5 ± 7 (7-29) years. Two patients had Williams Syndrome. The mean systolic pressure gradient of the supravalvular aortic stenosis of the patients evaluated echocardiographically and angiographically were measured 88 ± 36 mmHg (60-165 mmHg). The most seen associated cardiac anomally was subaortic stenosis (25%). The type of the supraaortic stenosis was local in all patients. We performed single sinus aortoplasty for correction the pathology.

Ther as no operative death and all of patients were alive. The median follow-up period was 62.25 ± 43.75 (12-146) months. All patients were followed up echocardiographically in the postoperative period. The mean residual gradient was 7.6 ± 7 mmHg (0-17 mmHg). No patients required reoperation and the actarial freedom from reoperation at 5 and 10 years after operation was 100%.

Supravalvular aortic stenosis is a progressive congenital disease and when it is not repaired in childhood, it causes left ventricular dysfunction and dilatation, tortuous or aneurysm at coronary arteries. Repair of supravalvular aortic stenosis by single sinus patch repair is safe and may be chose first because of good mid-long term hemodynamice results.

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