Ebstein心脏畸形外科矫治39例

Surgical correction of Ebstein's malformation in 39 cases

目的 总结Ebstein心脏畸形外科治疗经验。方法 回顾近 18年收治Ebstein畸形 39例 ,其中重型 8例 ,中间型 2 7例 ,轻型 4例。采用改良Danielson法矫正畸形 30例 ,其中 19例同时行瓣环成形 ,3例行瓣叶修补 ,6例行瓣交界成形 ;单纯三尖瓣成形 3例 ;Minale法修复 1例 ;双向上腔静脉—肺动脉连接和三尖瓣成形 1例 ;4例行三尖瓣置换。结果 2例 (成形和换瓣各 1例 )术后早期死于右心衰竭(5 1% )。成形术后三尖瓣功能正常 2 7例 (77 1% ) ,轻至中度关闭不全 5例 (14 3 % ) ,中至重度关闭不全 3例 (8 6 % )。生存者随访 0 5～ 18年 ,平均 8 6年。 33例心功能I级 ,3例心功能II级 (成形 2例、瓣膜替换 1例 ) ,1例行人工瓣置换者术后顽固性右心衰竭 ,于 1年后失访。结论 Ebstein畸形应尽早外科治疗。修复成形多能矫正畸形 。

Objective To review the experience of surgical treatment of Ebstein's malformation. Methods From February 1981 to May 1999, 39 patients with Ebstein's malformation underwent cardiac surgery. There were 8 cases with severe Ebstein's malformation, 27 moderate and 4 mild according to the Wang's criteria. The modified Danielson's procedure was applied in 30 patients. Concomitant procedures included annuloplasty in 19, leaflet repair in 3 and commissuroplasty in 6. Isolated tricuspid annuloplasty was performed in 3, Minole's technique in 1, bi directional cavopulmonary anastomosis and tricuspid plasty in 1, and tricuspid valve replacement in 4. Results There were 2 operative deaths with an operative mortality of 5.1%. the cause of death was right ventricular failure. After reconstructive operation, the function of tricuspid valve was normal in 27(79.4%), mild to moderate tricuspid regurgitation in 4(11.8%), and moderate to severe tricuspid regurgitation in 3(8.8%). Follow up ranged from 6 months to 18 years(mean 8.6 years). 3 patients are in NYHA class II (after repair in 2, replacement in 1). One case with tricuspid valve replacement had refractory right ventricular failure and lost after one year follow up. Conclusion Ebstein's malformation may be corrected by means of the repair and plasty, and bi directional cavopulmonary anastomosis is required when lesion is incorrigible.