先天性二尖瓣瓣上狭窄(附2例报告)

CONGENITAL SUPRAVALVULAR MITRAL STENOSIS: REPORT OF TWO CASES

本文报告2例二尖瓣瓣上狭窄,均于术前明确诊断并成功地进行了手术。该畸形与三房心类似,左房内有一膜状结构,位于瓣环上数毫米处,左心耳的下方,二者的根本区别在于心耳与膜状结构的位置关系。诊断依靠超声心动图和心血管造影。早期诊断的重要性在于倘若术前没有发现该畸形,术中仅将伴随畸形修补,将会导致严重后果。由于膜状结构距二尖瓣甚近,手术需要仔细操作,以免损伤二尖瓣。

Two cases of supravalvular mitral stenosis ring, a raer congenital cardiac deformity, are described in this paper. The diagnosis was confirmed preoper-atively and the operations were done successfully. This deformity resembles cor triatriatum, in which a membranous structure is present, usually a few millimeters above the annulus but below the aurical appendix. The distingui-shing feature between the two anomalies is the location of the atrial appendage in relation to the obstructing membrane. The diagnosis can be made by echocardiography and angiocardiography. The importance of early diagnosis of this lesion is evident. Failure to do so may result in a fatal outcome in the period following the operation for repair of associated anomalies only,because the membrane often closely attaches to the mitral valve. It requires a meticulous surgical technique to avoid injury to the mitral valve itself.