Sporadic supravalvular stenosis in a young man

Abstract

Supravalvar aortic stenosis (SVAS) is a form of congenital left ventricular outflow tract (LVOT) obstruction that occurs as a localized or diffuse narrowing of the ascending aorta beyond the superior margin of the sinuses of Valsalva. SVAS is a rare progressive congenital heart defect with a higher risk of sudden cardiac death. Our patient was a 30-yearold man referred by his GP for an incidental finding of heart murmur. Echocardiography showed a hypertrophic left ventricle with an increased pressure gradient. CT and MRI angiography and eventual arteriography confirmed the diagnosis by showing a narrowing of the aorta at the sinotubular junction with significant accelerated flow. This stenosis was eventually surgically corrected with no complications.