Radmehr Hassan, Salehi Mehrdad, Forouzan-Nia Seyed-Khalil, Emami Seyed-Ali, Mirhoseini Seyed-Jalil, Sanatkarfar Mehdi
Department of Cardiovascular Surgery, Tehran University of Medical Sciences, Tehran, Iran.
Arch Iran Med. 2006 Oct;9(4):354-8.
Ebstein anomaly is the downward displacement and adherence of dysplastic septal and posterior tricuspid leaflets into the right ventricle, thereby dividing the ventricle into a so-called atrialized chamber and a functionally reduced right ventricle. We evaluated the early and mid-term results of primary repair of Ebstein anomaly in adult patients.
Eight consecutive patients undergoing repair of Ebstein anomaly with Danielson technique at Imam Khomeini Medical Center, Tehran, Iran from January 1997 through July 2004 were evaluated. Functional and echocardiographic parameters were studied both preoperatively and postoperatively, as well as demographic status and adverse events.
Hospital mortality occurred in one patient because of right ventricular failure. The average follow-up period was 5.3 +/- 3.4 years (median: 3.8 years). The actuarial survival rate was 85.7 +/- 4.8% at 7 years. During the follow-up, six patients were in New York Heart Association functional class I, and one patient was in class II. None of the patients required reoperation related to their Ebstein repair. One patient demonstrated atrioventricular dissociation perioperatively; however, only one patient required permanent pacemaker insertion later. One patient had minimal (1+) regurgitation, with the jet localized along the anterior part of the ventricular septum. Two patients had residual tricuspid valve insufficiency (2+) on echocardiography.
Ebstein repair has a good functional and hemodynamic outcome in adult patients.
埃布斯坦畸形是发育异常的间隔和后叶三尖瓣叶向下移位并附着于右心室，从而将心室分为所谓的心房化腔室和功能减退的右心室。我们评估了成年患者埃布斯坦畸形一期修复的早期和中期结果。
对1997年1月至2004年7月在伊朗德黑兰伊玛目霍梅尼医疗中心连续8例接受丹尼尔森技术修复埃布斯坦畸形的患者进行评估。术前和术后研究功能和超声心动图参数，以及人口统计学状况和不良事件。
1例患者因右心室衰竭发生医院死亡。平均随访期为5.3±3.4年（中位数：3.8年）。7年时的精算生存率为85.7±4.8%。随访期间，6例患者纽约心脏协会心功能分级为I级，1例为II级。所有患者均无需因埃布斯坦修复相关原因再次手术。1例患者围手术期出现房室分离；然而，只有1例患者后来需要植入永久性起搏器。1例患者有轻微（1+）反流，反流束局限于室间隔前部。2例患者超声心动图显示有残余三尖瓣关闭不全（2+）。
埃布斯坦修复术在成年患者中具有良好的功能和血流动力学结果。
