主动脉瓣上狭窄:复杂病变的简单治疗

摘要

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.%主动脉瓣上狭窄(SAVS)是一种以升主动脉局限性或弥漫性狭窄为特征的先天畸形,可同时合并右心室流出道梗阻,主动脉瓣病变及冠状动脉开口狭窄.临床上,SAVS与Williams-Beuren综合征有很强的相关性,同时也表现为家族型或散发病例.通过学习国内、外文献,总结并探讨SAVS的临床表现、形态学、病因学及基因基础、手术治疗、手术早期效果、远期死亡及再次手术等.其病理生理的复杂性和影响转归的因素不仅在于主动脉瓣上的狭窄,是否合并其他畸形对治疗方案的选择至关重要.