[Supravalvular aortic stenosis. Apropos of 10 cases]

We present 10 patients diagnosed of supravalvular aortic stenosis (5 dumb bell shaped, 4 membranous and 1 undifferentiated), 7 in association with Williams-Beuren syndrome, 5 were male and 5 were female. None showed alterations of calcium metabolism and in 8 cases we found other associated cardiac anomalies. In 9, the diagnosis was confirmed with Eco-Doppler. The gradient calculated with the Eco-Doppler had a medium correlation (75%) with the catheterism (Doppler 46 +/- 23 (13-89)-Catheterism 27 +/- 15 (10-50)). 50% had the sinus of Valsalva dilated. Six patients underwent surgery, 5 with an enlargement parch and 1 with a butterfly parch. In 3 cases, associated anomalies were repaired, 2 during supravalvular surgery (one valvular aortic stenosis and one fixed subaortic stenosis). No patient had postoperatory complications.