Supravalvar aortic stenosis: simple procedure to a complex leision / 中华胸心血管外科杂志

ABSTRACT

Supravalvar aortic stenosis(SVAS) is a congenital anomaly characterized by a discrete or diffuse narrowing of the ascending aorta.It＇s also associated with right-ventricular outflow tract obstruction, aortic valve pathology and coronary ostial stenosis.Patients present in sporadic and familial forms, and may also associate with William-Beuren syndrome. In this article, the morphology, etiology, clinical presentation, genetic basis and various surgical approaches of SVAS are dis-cussed, along with an analysis of early results, associated procedures, late mortality and reoperation.Clinical-relevant patho-logic features other than the mere supra-aortic narrowing are diverse, have also important implications to surgical therapy.