Ebstein 畸形解剖纠治术

摘要

目的 总结20例Ebstein畸形解剖纠治术的近期效果。 方法 1997年12月至2000年9月， 对20例Ebstein畸形患者行解剖纠治术。其中男8例， 女12例，年龄3～32岁，平均11岁。Carpentier分型：A型2例，B型4例，C型14例。手术方法：梯形或三角形切除绝大部分房化右心室，沿瓣环切下下移的后瓣及其相连的乳头肌，必要时切下部分或全部隔叶，折叠缝合瓣环，使其恢复正常大小。将瓣叶根部移植于正常瓣环水平，并对瓣叶组织进行修复，重新移植或固定乳头肌，处理合并病变。合并手术包括：Rastalli手术1例、房间隔缺损修补术5例、缝闭卵圆孔4例、室间隔缺损修补术2例、动脉导管未闭直视缝闭术1例。 结果 患者术后均恢复顺利，心功能均恢复Ⅰ级。术前12例重度三尖瓣关闭不全患者，术后8例三尖瓣返流消失，4例三尖瓣轻度返流；8例中度关闭不全患者，术后7例三尖瓣返流消失，1例轻度三尖瓣返流；三尖瓣叶均在正常水平，房化心室基本消失。术后随访时间1～17个月，平均6个月。患者活动量恢复正常。超声心动图检查：17例三尖瓣返流消失，2例中度三尖瓣返流，1例轻度三尖瓣返流。 结论 Ebstein畸形解剖纠治术的近期效果良好，可能使绝大多数患者免于三尖瓣替换术，远期结果有待进一步观察。%Objective To anatomically correct Ebstein anomaly for therestoration of tricuspid and right ventricular function. Methods Between December 1997 and September 2000, twenty consecutive patients (8 male, 12 female; age range 3 to 32 years, mean 11 years) underwent anatomic correction of Ebstein＇s anomaly. Eleven patients had associated congenital malformation (atrial septal defect in 5 patients, patent foramen ovale in 4,ventricular septal defect in 2, patent ductus arteriosus in 1, and double outlet right ventricle in 1). Two patients were classified into Carpentier type A, 4 type B, and 14 type C. Operative techniques were trapezoid or triangle excision and suture atrialized chamber; shortening tricuspid annulus, detachment of the septal and (or) posterior leaflet from the displaced annulus and reattachment to the true tricuspid annulus; transection and reimplantation its or their papillary muscle; simultaneous correction congenital malformation. Results All patients survived and recovered well. Preoperative examinations revealed tricuspid incompetence (12 patients were severe, and 8 moderate). Postoperative echocardiography showed disappearance of tricuspid incompetence in 17 patients and mild tricuspid incompetence in 3. Their tricuspid valve leaflets were noted at the normal level, right ventricles reduced in size remarkably, and atrialized chamber vanished. Follow-up (1 to 17 months, mean 6 months) showed that their exercise tolerance returned to normal, and echocardiography revealed disappearance of tricuspid incompetence in 17 and mild in 3. Conclusion Our technique allows anatomic correction of Ebstein＇s anomaly with satisfactory early results, even in the cases usually reserved for primary valve replacement.