Isolated Diffuse Supravalvular Aortic Stenosis with Severe Aortic Narrowing in a 41-Year-Old Man

Isolated supravalvular aortic stenosis in adults is a rare form of left ventricular outflow tract obstruction. We describe a case in a 41-year-old man in whom the supravalvular aorta had narrowed to approximately the size of the left anterior descending coronary artery. The patient underwent aortic surgery with replacement of the ascending aorta and repair of supravalvular aortic stenosis with a pantaloon graft. A postoperative echocardiogram showed substantial improvement: the mean gradient across the aorta had fallen from 48 to 8 mmHg. Surgery is the definitive treatment in symptomatic patients with supravalvular aortic stenosis.