Sporadic supravalvular stenosis in a young man

Sporadic supravalvular stenosis in a young man

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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 Pumps 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 Oyster Knives 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.