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目的回顾室间隔缺损(VSD)远离2个大动脉开口的右室双出口(double_outlet right ventricle,DORV)的病理解剖特点、手术方法选择和治疗结果,对手术适应证和方法提出优化意见。方法1984年4月至2005年4月共有37例VSD远离2个大动脉开口的DORV患儿接受外科手术,年龄5个月~12岁,根据不同手术方法将病人分3组比较,其中心室内隧道修补术(intraventricular tunnel repair,IVR)组15例,Rastalli手术组10例,Fontan手术组12例。对限制性VSD,同时扩大VSD直径。结果手术住院死亡10例,死亡率27.0%(10/37例),其中1999年4月后死亡2例,死亡率10.5%(2/19)。IVR的手术死亡率(46.7%,7/15例)明显高于Rastalli组(20%,2/10例)和Fontan组(8.3%,1/12例)。结论三尖瓣与肺动脉之间距离是否大于主动脉瓣口直径是区别选择IVR手术和Rastalli手术的主要依据。共同房室瓣、三尖瓣附属组织跨越或嵌入到VSD和主动脉之间的区域、两个心室发育不平衡是我们选择改良Fontan的主要适应证。
OBJECTIVE: To review the pathological anatomy, surgical options and treatment outcomes of double-outlet right ventricle (DORV) with ventricular septal defect (VSD) far away from the two major arteries and to make suggestions on surgical indications and methods. Methods From April 1984 to April 2005, a total of 37 children with DORV who had VSDs far away from the two major arteries were aged from 5 months to 12 years old. According to different surgical methods, the patients were divided into three groups. The central tunnel There were 15 cases of intraventricular tunnel repair (IVR), 10 cases of Rastalli and 12 cases of Fontan. For restricted VSD, expand VSD diameter at the same time. Results The hospitalized patients were killed in 10 cases, with a mortality rate of 27.0% (10/37 cases). Among them, 2 were killed after April 1999 and the mortality rate was 10.5% (2/19). Surgical mortality of IVR (46.7%, 7/15 cases) was significantly higher than that of Rastalli group (20%, 2/10 cases) and Fontan group (8.3%, 1/12 cases). Conclusion Whether the distance between the tricuspid valve and the pulmonary artery is greater than the diameter of the aortic valve orifice is the main basis for the difference between IVR and Rastalli. Common atrioventricular valve, tricuspid valve attachment spanning or embedding in the area between the VSD and the aorta, and two ventricular dysplasia are the main indications for our choice of modified Fontan.