Ebstein心脏畸形24例的外科矫治

Surgical correction of Ebstein’s anomaly in 24 cases

目的总结24例Ebstein畸形患者的外科手术治疗经验。方法回顾性分析2002年9月至2009年5月24例Ebstein畸形患者手术治疗的临床资料,采用Danielson水平折叠法矫治畸形17例,其中同时行瓣环成形12例,瓣交界成形5例,瓣叶修补4例;采用Carpentier纵行折叠5例,均同时行瓣环成形;1例采用解剖矫治;1例行三尖瓣置换术。合并畸形同期处理。结果全组无死亡,无严重心功能不全和顽固性低心排出量综合征发生,无因三尖瓣病变再次手术者。随访2～81个月,心功能Ⅰ级15例,Ⅱ级7例,2例恢复稍差,心功能Ⅱ～Ⅲ级。结论 Ebstein畸形应尽早外科矫治,外科治疗应根据每例患者瓣膜和心室的具体条件选择恰当的术式,恰当的瓣膜成形或瓣膜置换可以取得较好的中、远期效果。

Objectives To summarize the experience of corrective surgery for Ebstein＇s anomaly. Methods We retrospectively analyzed 24 patients with Ebstein＇s anomaly who were operated on from September 2002 to May 2009. The Danielson＇s procedure was applied in 17 patients. Concomitant procedures included annuloplasty in 12 patients, commissuroplasty in 5 patients and leaflet repair in 4 patients. Carpentier＇s procedure was applied in 5 patients with concomitant annuloplasty. Anatomic correction and tricuspid valve replacement were performed in 1 patient respectively. Complicated abnormalities were corrected at the same time. Results There was no death, and no severe heart dysfunction and refractory low cardiac output in all postoperative patients. No reoperation for residual or recurrent tricuspid incompetence was required in all patients. At follow-up of all patients ranging from 2 to 81 months, 15 patients were in New York Heart Association （NYHA） class I , 7 in class Ⅱ , 2 in class Ⅱ -Ⅲ. Conclusions Ebstein＇s anomaly should be surgical corrected as early as possible. The selection of surgical procedures should be based on the pathological anatomy of tricuspid valve and function of ventricular. Appropriate valve plasty or replacement allows surgical correction of Ebstein ＇ s anomaly with satisfactory medium and long-term results.