Ebstein畸形的外科治疗进展

The Advance of Surgical Treatment of Ebstein Anomaly

Ebstein畸形是一种较少见的先天性心脏畸形,可明显影响患者的心功能,随着病情的进展最终诱发右心衰竭甚至全心衰竭,而导致患者死亡。近年来,Ebstein畸形手术主要在针对病变瓣膜的处理、右心室功能完善及相关并发症的预处理上已取得较大的进展。但由于其病理解剖及临床表现的复杂多样性,目前尚未有一个"金标准"来治疗所有病理解剖类型的畸形患者,也一直未有一种手术策略来解决临床所遇到的实际问题,所以术后疗效不尽如人意。我们通过文献复习,对Ebstein畸形的外科治疗进展及其相关并发症防治等进展进行综述。

Ebstein anomaly is a relatively rare congenital heart malformation which can affect heart function significantly.It may cause right heart failure,even whole heart failure and eventually death.In recent years,the surgery has made much progress in dealing with the abnormal valve,improving the right ventricle function and pretreatment of its related complications.However,because of its complexity and diversity in pathological anatomy and clinical manifestations,the disease has not got an ＂almighty standard＂ to treat all pathological anatomy types of the deformity,or a surgery strategy to solve the practical problems encountered in all clinic situations.Furthermore,the therapeutic effect is also unsatisfactory.This article will review the advance of treatment of Ebstein anomaly and prevention of its related complications.