手术治疗先天性三尖瓣下移畸形62例临床分析

Surgical treatment of Ebstein's anomaly in 62 cases

目的探讨先天性三尖瓣下移(Ebstein)畸形的手术治疗方法、效果及安全性。方法 62例Ebstein畸形患者,1例Carpentier分型D型行双向Glenn术,44例(Carpentier分型A型6例、B型34例、C型4例)行改良Carpentier法三尖瓣成形术,17例(Carpentier分型B型2例、C型15例)行改良Carpentier法三尖瓣成形术+双向Glenn术;合并室间隔缺损、肺动脉狭窄、部分性心内膜垫缺损、动脉导管未闭者均同期进行矫治;房间隔缺损或卵圆孔未闭者视三尖瓣瓣口面积和右心房压力决定是否保留部分或全部房间隔或卵圆孔。记录手术情况,随访观察手术治疗效果。结果手术体外循环时间61~142min,主动脉阻断时间31~102min,术后呼吸机辅助时间5h^24d,ICU住院时间1~40d;围术期死亡3例,余59例术后6~106d出院,随访1个月~6a,心功能及活动能力正常,均未出现中度以上三尖瓣反流和心律失常等并发症。结论采用改良Carpentier法三尖瓣形成术和/或双向Glenn术矫治Ebstein畸形,解剖学上可获得满意的纠正,术后远期效果优良。

Objective To summarize the method,outcome and safety of surgical treatment of Ebstein's anomaly.Methods Among 62 cases of Ebstein's anomaly,1 case of Carpentier type D underwent Glenn procedure,44 cases(6 of type A,34 of type B,4 of type C)underwent tricuspid valve repair with modified Carpentier procedure,and 17cases(2 of type B and 15 of type C)underwent modified Carpentier plus Glenn procedure.The ventricular septal defect,pulmonary artery stenosis,partial endocardial cushion defect and patent ductus arteriosus were repaired simultaneously.Whether to remain or partially close the atrial septal defect or patent foramen ovale was decided according to the area of tricuspid valve and the pressure of right atrium.The operation process was recorded and the outcome of surgical treatment was followed up.Results The cardiopulmonary bypass lasted for 61 to 142 min and the aortic cross clamping lasted for 31 to 102 min.The mechanical ventilation lasted for 5h to 24 d and the length of stay in intensive care unit was 1 to 40 d.There were 3 early hospital mortalities.Other 59 survived patients stayed in hospital for 6 to 106 days and were followed up for 1 month to 6 years.The cardiac function of these 59 patients was normal,and no severe tricuspid regurgitation or other complications such as cardiac arrhythmia was found.Conclusion Ebstein's anomaly can be anatomically corrected with modified Carpentier procedure with an excellent long-term effect.