Ebstein畸形外科治疗30例临床分析

Analysis of surgical treatment of Ebstein anomaly in 30 cases

目的探讨Ebstein畸形的手术方法及疗效。方法总结30例手术治疗Ebstein畸形患者的临床资料,男10例,女20例;年龄3～48岁,平均(17±11)岁;术前心功能Ⅰ级10例,Ⅱ级14例,Ⅲ～Ⅳ级6例。超声心动图提示三尖瓣重度反流16例,中度反流9例,轻度反流5例。全组2例行三尖瓣置换术,其余均行三尖瓣成形术;3例采用Danielson法,25例采用Carpentier法,其中6例行一个半心室矫治术。合并畸形同期矫治。结果无早期手术死亡病例。术后复查仍有轻度三尖瓣反流16例。随访1个月～14年,除2例三尖瓣反流加重并心功能进行性下降行三尖瓣置换术外,其余三尖瓣反流减轻。结论采用Carpentier法施行三尖瓣成形可明显减轻三尖瓣反流,保护右心功能。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 discuss and evaluate operative methods and therapeutic efficacy of Ebstein anomaly. Methods Thirty cases of Ebstein anomaly underwent operation during May 1996 to May 2009 were collected. Ten were male and 20 were female, their age ranged from 3 years to 48 years with a mean age of （17±11） years old. Ten patients were classified as New York Heart Association （NYHA）class Ⅰ , 14 were class Ⅱ and 6 in class Ⅲ-Ⅳ. The tricuspid regurgitation were severe in 16 patients by echocardiogram, while 9 patients were moderate and 5 patients were mild. Two patients had mechanic valve replacement. Twenty-eight patients had tricuspid valve repairment, 3 patients were operated with Danielson＇s technique and 25 patients were operated with Carpentier＇s technique. Six patients were performed one and a half ventrieular repairment. The complicated heart lesions were corrected at the same time. Results No patient died. There was light tricuspid regurgitation in 16 patients postoperatively. All were followed from 1 month to 14 years. Tricuspid regurgitation was improved except in 2 patients who underwent mechanic valve replacement because tricuspid regurgitation were much severer. Conclusions Tricuspid valve repairment can evidently relieve regurgitation. Whether one and a half ventricular repairment should be adopted can be determined by the function of tricuspid valve and right ventricle.