室间隔缺损远离大动脉开口的右室双出口外科治疗与适应证选择  

Surgical managements and their indications of double-outlet right ventricle with non-committed ventricular septal defect

作　　者：王顺民[1] 徐志伟[1] 刘锦纷[1] 曹鼎方[1] 史珍英[1] 朱德明[1] 苏肇伉[1] 丁文祥[1] 

机构地区：[1]上海交通大学医学院附属新华医院上海儿童医学中心小儿心胸外科,上海200127

出　　处：《临床儿科杂志》2005年第12期841-844,共4页Journal of Clinical Pediatrics

基　　金：上海市科委科研基金资助项目(004119083)

摘　　要：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 anatomy features, the surgical managements and the results of the patients with double-outlet right ventricle （DORV） accompanying non-committed ventricular septal defect （VSD） and optimize the surgical strategies and managements for them. Methods Thirty-seven cases （aged from 5 months to 12 years） with DORV with non-committed VSD received surgical therapy （from April, 1984 to April, 2005） . They were divided into three groups based on different surgical managements. Group IVR （intraventricular tunnel repair, 15 cases） ; Group Rastelli（10 cases） ; Group Fontan （12 cases） .Some restrictive VSDs were enlarged during operations. Results The whole hospital mortality rate was 27.0% （10/37）. Hospital mortality rate decreased to 10.5% （2/19）since April 1999. The hospital mortality rate in group IVR （46.7 % ,7/15）was higher than that in group Rastelli （20 %, 2/10） and in group Fontan （8.3 %, 1/12）. Conclusions Whether the tricuspid pulmonary distance is larger than the diameter of the aorta is the key consideration in selecting IVR and Rastelli operations. Common atrial-ventricular valve, the interference of the tricuspid valve and its apparatus between the VSD and the aorta, and the imbalance of the two ventricles were the major indications in choosing Fontan operation.

关 键 词：右室双出口 心脏手术 适应证 

分 类 号：R726.5[医药卫生—儿科]