45例成人Ebstein畸形的外科治疗

摘要

Objective To review retrospectively the experience of surgical treatment of Ebstein anomaly in 45 adult patients.Methods Between January 2008 and December 2011,45 adult patients underwent surgery for Ebstein anomaly.There were 14 male and 31 female patients aged 18 to 61 years (mean,39 years).The main symptoms included chest tightness,shortness of breath,palpitation.25 patients had cyanosis,while 6 patients with Wolff-Parkinson-White syndrome.Preoperative echocardiography: The septal downward from 2.5 cm to 7.5 cm,average 4.2 cm; the posterior leaflet downward 3.0 cm to 8.0cm,average 5.1 cm.Tricuspid incompetence was moderate in 6 patients and severe in 29.According to Carpentier classification,5 cases of type A,30 type B,type C in 6 cases.Preoperative New York Heart Association(NYHA) functional class Ⅱin 14 cases,Ⅲ 30 cases,Ⅳ in 1 cases.Surgical procedures included modified Danielson's in 15 patients,modified Carpentier's in 23,and 7 patients underwent tricuspid valve replacement(incloud 4 secondary operations).The ratio of tricuspid valve repair was 84.4％.Autologous pericardium patch was used to reconstruct the septal and posterior leaflets in 5 cases.Intraoperative use of annuloplasty rings in 21 cases.Results There were 2 hospital deaths with a postoperative mortality of 4.4％.The main causes of death were low cardiac output and right heart failure.Operation to discharge time was (8.0 ± 3.8)days.The follow up time was 2 to 46 months.Echocardiography examination: Within the 32 patients of severe tricuspid regurgitation before tricuspid valve plasty,16 patients’ tricuspid regurgitation disappeared,13 patients with mild regurgitation,3 moderate regurgitation.Within 5 patients of moderate tricuspid regurgitation before tricuspid valve plasty,4 cases tricuspid regurgitation disappeared after operation,1 case tricuspid valve mild regurgitation.Tricuspid valve leaflets were at normal levels,the atrialized portion of the ventricular disappeared.The heart function improved to be NYHA class Ⅰ to Ⅱ in 95 ％ of the survived patients.Conclusion Operative method of Ebstein anomaly sould be selected according to the type of pathoanatomies.Tricuspid valve repair should be performed to the best of our ability.%目的 回顾分析45例成人Ebstein畸形外科手术方法及疗效.方法 45例Ebstein畸形患者,男14例,女31例;年龄18 ～61岁,平均39岁.行改良 Carpentier 术23例,改良Danielson术15例,三尖瓣置换术7例(二次手术4例).结果 三尖瓣成形术后超声心动图示三尖瓣反流明显减轻.早期死亡2例(4.4％),死因为术后低心排血量综合征和右心功能衰竭.随访2～ 46个月,生存者中41例(95％)恢复心功能Ⅰ～Ⅱ级.结论 根据Ebstein畸形的病变程度选择合适的手术方法是手术成功的关键.手术方式要个体化,尽量行成形术.