Supravalvar aortic stenosis in children report of thirteen cases

ABSTRACT

During 15 years from 1975 to 1990, thirteen cases of supravalvar aortic stenosis were admitted at the pediatrics department of Shahid Rajai Heart Hospital, Tehran. All patients were subjected to cardiac catheterization and an angiocardiography. Patients ranged in age from 3.5 years to 14 years with a mean of 8.7 years. Seventy seven percent of children were male. Eight cases [61.5%] had Williams' syndrome. Hourglass type of supravalvar aortic stenosis was the most common variant [70%], followed by hypoplastic type [15%] and membranous type [5%]. Peripheral pulmonary stenosis was the most common associated anomaly and was observed in 23% of patients. The main purpose of this investigation is to report the results of the study on supravalvar aortic stenosis and its association with Williams' syndrome in a group of Iranian children