Isolated supravalvular aortic stenosis with left ventricular diverticulum and cleft mitral valve: Surgical repair in adulthood

Abstract

Supravalvular aortic stenosis is an uncommon but well characterized congenital narrowing of the ascending aorta above the level of the coronary arteries. It can be a familial disorder, can occur sporadically, or can be associated with Williams syndrome. We are reporting a very rare presentation of supravalvular aortic stenosis with associated left ventricular diverticulum and cleft mitral valve. Repair consisted of resection of the ascending aorta, patch augmentation of the aortic root, and mitral valve repair. Follow-up echocardiography demonstrated normal mitral and aortic valve function and a postoperative three-dimensional computed tomographic scan showed a normal shape of the reconstructed ascending aorta.

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

“…2 Our patient had isolated SVAS, which is rare; only a few cases have been reported. [4][5][6][7] He did not have Williams syndrome, and he had no other conditions that predisposed him to SVAS.…”