Ebstein 心脏畸形的临床解剖及外科治疗

目的总结先天性三尖瓣下移畸形（Ebsein 畸形）的临床解剖分型及外科治疗方法。方法2005年7月到2009年3月收治的Ebstein 畸形25例患者，根据术中所见的三尖瓣畸形及下移的程度分为Ⅰ型8例，Ⅱ型12例，Ⅲ型5例。其中Ⅰ型全部行后瓣环成形术，Ⅱ型中9例行房化心室折叠术+三尖瓣成形术，2例行三尖瓣置换术，1例行三尖瓣成形+双向腔肺动脉分流术（双向Gleen术）。Ⅲ型全部行三尖瓣置换术。结果全组病例无死亡。2例术后出现低心排综合征，应用强心利尿药后均好转，其余患者术后恢复良好。随访15例,随访时间4个月～2年，复查超声心动图显示：12例三尖瓣水平无返流，3例三尖瓣水平见轻-中度返流信号，10例心功能Ⅰ级(NYHA分级)，5例心功能Ⅱ级（NYHA分级）。结论Ebstein 心脏畸形的临床病理解剖分型对其临床诊断及手术方案的选择有较高的价值。

Clinical anatomy and surgical treatment of Ebstein′s anomaly

Objective To summarize the clinical anatomic features and surgical treatment results of Ebstein's anomaly. Methods 25 patients with Ebstein's anomaly were operated on from June 2005 to January 2009. There were 8 type 1 cases, 12 type Ⅱ cases, and 5 type Ⅲ cases. The type Ⅰ group received tricuspid valve plasty( Devega tricuspid annuloplasty). In the type Ⅱ group, 9 cases received atrialized ventricle plication and tricuspid valve plasty, 2 cases tricuspid valve replacement,and 1 case tricuspid valve plasty and Gleen. The type Ⅲ group received tricuspid valve replacement. Results There were no operative deaths. Low cardiac output occurred in 2 patients after the operation, but they quickly recovered after proper treatment. 15 patients were followed up from 4 months to 2 years, and all had echocardiography. There was no reflow of the tricuspid valve in 12 patients and light reflow in 3 patients. 10 patients were in NYHA Class 1.5 patients were in NHYA Class Ⅱ. Conclusion Accurate pre-op-erative diagnosis and full understanding of patho-physiology and clinical anatomy are the keys for appropriate surgical treatment.