Ebstein畸形的外科治疗

Surgical treatment of Ebstein anomaly

目的总结Ebstein畸形的手术治疗经验，以提高临床疗效。方法手术治疗Ebstein畸形35例，NYHA心功能分级Ⅰ级11例，Ⅱ级14例，Ⅲ-Ⅳ级10例；超声心动图示：三尖瓣重度反流24例，中度反流5例，轻度反流6例。2例行三尖瓣置换术；33例行三尖瓣成形＋房化心室折叠术，采用Danielson法2例，Carpentier法31例，其中9例行一个半心室矫治术。合并畸形同期矫治。结果术后死亡1例；三尖瓣置换术后发生第三度房室传导阻滞2例，低心排血量综合征2例，心房颤动合并频发室性期前收缩2例。出院前复查胸部X线片和超声心动图，提示心脏缩小，三尖瓣反流消失22例，轻度反流9例，中度反流3例。术后随访1个月至7年，21例患者三尖瓣反流消失；12例患者存在轻度三尖瓣反流，心功能Ⅰ～Ⅱ级；1例三尖瓣反流加重，心功能不全，于术后3年行三尖瓣置换术，心功能恢复至Ⅰ～Ⅱ级。结论Ebstein畸形是一种少见的先天性心脏病，采用Carpentier法施行三尖瓣成形术效果良好；对三尖瓣和右心室发育不良者施行一个半心室矫治术有利于右心功能改善。

Objective To summarize the experience in surgical treatment of Ebstein anomaly, and evaluate the therapeutic effect. Methods Thirty-five patients of Ebstein anomaly were treated by operation. New York heart association （NYHA） heart function class Ⅰ was in 11 cases,class Ⅱ was in 14 cases,class Ⅲ-Ⅳ was in 10 cases. The cue of echocardiogram about tricuspid valve regurgitation in 24 cases were severe, 5 cases were moderate and 6 cases were mild. Two patients accepted tricuspid valve replacement. Thirty-three cases were accepted tricuspid valve repair and right ventricle folded, in which 2 cases accepted Danielson method, and 31 cases accepted Carpentier method, among them, 9 cases accepted one and a half ventricular repair. Associated heart anomaly was corrected at the same time. Results One patient died. There were 2 cases with third degree atrioventricular block after tricuspid valve replacement, 2 cases with low cardiac output syndrome and 2 cases with atrial fibrillation combined premature ventricular contraction. The heart was lower by chest X-ray and echocardiogram examination, tricuspid valve regurgitation in 22 cases were disappeared, 9 cases were mild, 3 cases were moderate. All patients were followed up from 1 month to 7 years, tricuspid valve regurgitation in 21 cases were disappeared, 12 cases were mild,heart function were class Ⅰ-Ⅱ. Because of heart function aggravation, 1 patient was operated again 3 years after operation. Conclusions Ebstein anomaly is a rare congenital heart disease,Carpentier method tricuspid valve repair can decrease regurgitation obviously and protect right ventricular function. One and a half ventricular repair should be adopted according to the function of tricuspid valve and right ventricle.