室间隔完整型肺动脉闭锁的外科治疗

Surgical Repair of Pulmonary Atresia with Intact Ventricular Septum

目的探讨室间隔完整型肺动脉闭锁(PA-IVS)的最佳外科治疗方法.方法对2001年1月至2004年6月北京儿童医院心脏中心收治的11例PA-IVS患儿进行回顾性研究.患儿年龄34 d至2岁8月,平均(8.00±9.04)月;体质量4.2～10 kg,平均(6.67±1.86)kg.合并畸形:11例均合并动脉导管未闭及房间隔缺损或卵圆孔未闭,右心室流出道肥厚性狭窄10例,右心室发育不良合并三尖瓣重度狭窄2例.手术方法:非体外循环下肺动脉瓣切开扩张+动脉导管结扎术1例;低温或浅低温体外循环下行右心室-肺动脉干重建术+动脉导管闭合、房间隔缺损修补术8例;Glenn手术1例;Fontan手术1例.结果术后死亡2例(18.2%),此2例均有严重的右心室发育不良,其中1例行右心室流出道疏通+加宽术,1例行Fontan手术,均死于右心衰竭.8例获得随访,时间1月至3年,平均为13月.8例患儿经皮氧饱和度均大于0.90.肺动脉瓣轻度狭窄1例;右心室流出道轻度狭窄1例.结论 PA-IVS一旦诊断应尽快手术治疗;手术效果与右心室发育程度有密切关系.

Objective To investigate a more effective surgical approach to treat pulmonary atresia with intact ventricular septum (PA-IVS). Methods From January 2001 to June 2004, 11 patients with PA-IVS were enrolled in a retrospective study, aged from 34 d to 32 month old, averaged (8.00±9.04) months, with body weight from 4.2 kg to 10 kg, (6.67±1.86) kg. All of 11 patients complicated with PDA and ASD/PFO, 10 patients with RVOTO, 2 patients with right ventricle or tricaspid valve hypoplasia. One patient received pulmonary valvotomy without CPB, and eight patients had transventricular pulmonary valvotomy with patch repaired, one patient received operation for Glenn shunt, and the other one had Fontan procedure. Results The early post-operation mortality was 18.1% (2 cases), and the 2 cases were complicated with severe right ventricular, one case had hypoplasia pulmonary valvotomy and enlarged right ventricle with Gore-tex patch, the another one had Fontan procedure. Eight patients were followed-up for 1 month to 3 years; the blood O 2 saturation of eight patients was over 0.9. Two cases remained in mild stenosis of the pulmonary valve or in the right ventricular outflow tract. Conclusion PA-IVS patients should be surgically repaired as early as possible and the surgical results depends on the growth of right ventricle and tricuspid valve.