Ebstein心脏畸形139例手术治疗经验

目的：总结Ebstein心脏畸形手术治疗的经验。方法：回顾性分析1980年6月至2000年1月手术治疗139例Ebstein心脏畸形病人。其中行三尖瓣成形、房化右心室折叠和三尖瓣环DeVega成形111例，三尖瓣置换术27例，右心室发育不全行心外管道全腔静脉-肺动脉连接术（ETCPC）1例。结果：全组手术死亡12例（8.6％）；近10年手术死亡率降至3.3％。成形组中10例再次行瓣膜置换术，均生存。结论：对Ebstein心脏畸形应根据其美丽解剖特征选择手术方案，轻型可选用三尖瓣成形术；中间A型可选用成形术，中间B型应慎重选择成形或瓣膜置换术；重型选用瓣膜置换术。

Experience in corrective surgery for Ebstein's anomaly of 139 cases

Objective: To summarize the experience of corrective surgery for Ebstein's anomaly. Methods: 139 patients with Ebstein's anomaly were operated on from June, 1980 to January, 2000. Among them, 111 cases received atrialized ventricle plication, tricuspid valve plasty and Devega tricuspid annuloplasty, 27 cases undergoing tricuspid valve replacement. One case of right ventricular hypoplasia received extra total cavopulmonary connection (ETCPC). Results: In the whole group, there were 12 operative deaths, with mortality rate of 8.6%. While in the later 10 years, operative mortality rate was reduced to 3.3%. In the group of plastive operation, 10 cases were re-operated with valve replacement and all were survived. Conclusion: Operative method of Ebstein's anomaly should be decided according to the pathologicoanatomic feature. For mild type, tricuspid valve plasty should be chosen. For medium A type, plastic operation should be performed. For medium B type, plasty or valve replacement should be cautiously selected. For severe type, valve replacement should be considered.