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先无性右室双出口(Double OutletRight Ventricle,DORV)是相对少见的紫绀型先天性心脏病。主要畸形是二支大血管均发自右心室或一支完全发自右心室,另一支主要发自右心室(至少80％以上),而左室的唯一出口是同时存在的室间隔缺损。根据二支大血管的位置关系及室间隔缺损(VSD)的部位可有16种解剖结构的变异。 本症于1949年由Taussig和Bing首先报告,但未应用DORV之名;其主要畸形是主动脉(AO)及肺动脉(PA)并排发自右室,VSD位于肺动脉下为嵴上型,大动脉氧含量PA O_2>AO O_2,实际上为DORV的Taussig—Bing型。1957年Witham总结文献6例并加上自己的4例第一次应用了
Double OutletRight Ventricle (DORV) is a relatively rare cyanotic congenital heart disease. The main deformity is that the two major vessels are all from the right ventricle or one from the right ventricle and the other from the right ventricle (at least 80%), while the only exit to the left ventricle is the simultaneous presence of ventricular septal defects. According to the location of the two large vessels and ventricular septal defect (VSD) site there may be 16 kinds of anatomical variations. The disease was first reported by Taussig and Bing in 1949, but no DORV name was applied; its major deformities were aorta (AO) and pulmonary artery (PA) side-by-side from the right ventricle, VSD below the pulmonary artery for crista, aorta PAO 2> AO O 2, actually TORSOIG-Bing of DORV. In 1957 Witham concluded 6 articles and added his own 4 cases for the first time