Peripheral Vascular Anomalies Associated with the Supravalvular Aortic Stenosis Syndrome

Abstract

On the basis of facial characteristics, 15 patients with supravalvular aortic stenosis syndrome were divided into 3 clinical groups, A, B, and C. Patients in Groups A and B suffered moderately severe mental retardation; in those in Group C the I.Q. was normal. Diagnostic cardiac catheteriz-ation, cine angiocardiography, and aortography demonstrated multiple cardiovascular abnormalities in patients in each of the 3 groups: pulmonary stenosis in 8; supravalvular aortic stenosis in 14; coronary artery abnormalities and hypoplasia of the thoracic aorta in 9; celiac and mesenteric artery stenoses in 3. Stenosis or atresia of one of the brachiocephalic vessels was found in 1 patient, and renal stenosis in 3. Biplane serial radiography was superior to cineradio-graphy in evaluation of vascular lesions of the brachiocephalic vessels.