1(1/2)心室修补手术治疗Ebstein畸形

Surgical treatment of Ebstein's anomaly with one-and-one-half ventricular repair

目的 总结 1 心室修补手术治疗Ebstein畸形的经验。方法 1997年 1月至 2 0 0 2年 8月对 9例中、重度Ebstein畸形病人 ,行 1 心室修补手术 ,其中合并房间隔缺损 (右向左分流 ) 5例 ,三尖瓣为轻至重度反流 ;所有病人术前均有症状 ,其中心功能Ⅱ级 5例 ,心功能Ⅲ级 4例。除行三尖瓣成形及闭合房间隔缺损外 ,均行上腔静脉右肺动脉双向分流术。结果 本组无手术死亡。 1例 5岁重度三尖瓣下移患儿术后出现右侧胸腔积液 ,经穿刺治疗半月后胸腔积液消失。随访 3个月至 5年 ,心功能恢复到Ⅰ级 ,复查心脏超声提示三尖瓣轻度反流。结论 1 心室修补手术治疗可能是治疗中、重度Ebstein畸形较好的方法。其远期疗效有待于进一步观察。

Objective To summarize the experiences in the surgical treatment of Ebstein's anomaly with one and one half ventricular repair. Methods From January 1997 to August 2002, 9 patients with moderate or severe Ebstein's anomaly and mild to severe tricuspid regurgitation underwent the one and one half ventricular repair. Of the 9 patients, 5 were associated with atrial septal defects(ASD) with right to left shunt. All patients were symptomatic in New York Heart Association Functional Class Ⅱ( n =5) or Ⅲ( n =4). In addition to tricuspid valve repair and closure of ASD, all patients underwent bidirectional cavopulmonary shunt(BCPS). Results There were no surgical deaths. Pleural effusions occurred in a 5 year old patient with severe Ebstein's anomaly but disappeared 15 d after paracentesis. New York Heart Association Functional Class I and trivial tricuspid regurgitation were found in all patients after a follow up ranging from 3 months to 5 years. Conclusion The one and one half ventricular repair may be an alternative approach to the management of moderate or severe Ebstein's anomaly. The long term curative effect of the surgical procedure needs to be further studied.