Supravalvular aortic stenosis

Abstract

Congenital supravalvular aortic stenosis is an uncommon condition which includes several morphological entities. Patients have a prominent systolic thrill and systolic ejection murmur without an ejection click, maximally over the carotid arteries and in the suprasternal notch, as well as along the right upper sternal border. On telecardiograms, absence of poststenotic dilatation of the ascending aorta should give rise to suspicion. Cardiac catheterization demonstrates a pressure gradient just above the aortic valve. Left ventricular or aortic root angiography indicates the degree of obstruction and provides additional information concerning the ascending aorta and its branches. In this paper, a rare hourglass type supravalvular aortic stenosis with normal facies and intelligence is presented. The patient was operated on utilizing cardiopulmonary bypass and resection of the obstruction, and patch plasty at the narrowed site of the aorta was successfully performed. This procedure relieves the high pressure on the coronary arteries and improves the left ventricular function, so that operation is advised as soon as possible after the diagnosis is confirmed.