三尖瓣下移畸形237例手术治疗结果分析

The outcomes of operation for 237 patients with Ebstein anomaly

目的探讨三尖瓣下移畸形外科治疗的近期和远期随访结果。 方法回顾性分析2004年3月至2017年12月清华大学第一附属医院心外科收治的237例三尖瓣下移畸形患者的临床资料及随访结果。男性105例，女性132例；年龄（19.4±16.7）岁（范围：3个月至64岁）。237例患者共接受了242例次手术，包括解剖矫治术188例，一个半心室功能矫治术37例，三尖瓣成形术4例，三尖瓣置换术10例，全腔静脉－肺动脉吻合术2例，双向Glenn手术1例。 结果手术早期死亡5例（2.1%），术后发生Ⅲ度房室传导阻滞1例（0.4%）。228例获得随访，随访率为97.9%，随访时间3～168个月，2例（0.9%）远期死亡。患者三尖瓣反流指数由术前3.6±0.3降至术后1.5±0.4。患者术后5年、10年累积生存率分别为98.6%、98.2%。 结论三尖瓣下移畸形手术治疗应尽可能以正常的三尖瓣及右心室结构为标准进行解剖矫治，可以获得良好的治疗效果。应以个体化治疗为原则，对病情极为严重的患者进行必要的一个半心室功能矫治和瓣膜置换。

ObjectiveTo evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly. MethodsThe clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of （19.4±16.7） years （ranging from 3 months to 64 years）. The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients （total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient）. ResultsThe early mortality was 2.1% （n=5）. One case of atrioventricular （0.4%） newly occurred. There were 228 patients available to follow-up. The range of follow-up duration was 3 to 168 months. Late survival was 99.1% （2 cases of late death） at 10 years. Three patients received reoperation （1.3%）, including tricuspid valve repair of 1 patient and one and a half ventricle repair of 2 patients）. Indication of tricuspid valve regurgitation improved from 3.6±0.3 to 1.5±0.4. Survival rate at 5 and 10 years was 98.6% and 98.2%, respectively. ConclusionsThe principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. For most cases, the anatomical repair was demonstrated with low mortality, less complications and excellent durability at long-term follow-up. If the tricuspid valve is severely hypoplastic, one and a half ventricle repair and valve replacement may be alternatie.