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目的探讨和评价小儿肺动脉闭锁(PA)伴室间隔缺损(VSD)右室流出道重建方法的应用。方法1999年4月至2004年12月,收治81例PA伴VSD行一期或分期的双心室修补术病儿。一期根治术组32例,大部分病例采用自体心包片跨瓣环水平扩大右室流出道至肺总动脉,其中4例带心包单瓣,合并主肺动脉侧支(MAPCAs)的11例中有2例完成肺动脉血管的单元化手术。姑息手术组49例,分别采用不同的右室流出道重建方法。其中15例完成了二期根治术,大部分采用同种异体心包片跨瓣补片。结果一期根治术死亡4例(12.5%);姑息手术死亡3例(6.1%);二期根治术无死亡。出院时根治术组经皮氧饱和度0.95~0.98。随访3~24个月。姑息手术为0.79~0.87,McGoon指数从0.4~0.7增至1.1~1.6。结论术前判断肺动脉干、肺动脉分支发育情况、中央共汇、有无MAPCAs,对手术方法的选择极为重要。术后PRV/PLV的测定有助于右室流出道重建方法预后的评估。
Objective To investigate and evaluate the application of reconstruction of right ventricular outflow tract in children with pulmonary atresia (PA) and ventricular septal defect (VSD). Methods From April 1999 to December 2004, 81 patients with PA or VSD underwent one-stage or staged biventricular surgery. In the first stage, 32 cases were treated with autologous pericardium, and the right ventricular outflow tract was enlarged to the common pulmonary artery through the annulus. Among them, 4 cases had pericardial single flap and 11 cases had merger of the main pulmonary arteries (MAPCAs) 2 cases completed the pulmonary artery unitation surgery. Palliative surgery group of 49 cases, respectively, using different methods of right ventricular outflow tract reconstruction. Among them, 15 cases completed the second stage radical mastectomy, most of them using allogenic pericardial transpedicular flap. Results In the first stage, 4 cases died of radical surgery (12.5%), 3 cases died of palliative surgery (6.1%), and none died in the second stage. Radical surgery at discharge group percutaneous oxygen saturation 0.95 ~ 0.98. Follow up for 3 to 24 months. Palliative surgery was 0.79 to 0.87, McGoon index increased from 0.4 to 0.7 to 1.1 to 1.6. Conclusion Preoperative determination of pulmonary artery and pulmonary artery branch development, the central common exchange, with or without MAPCAs, the choice of surgical approach is extremely important. Postoperative determination of PRV / PLV contributes to the assessment of the prognosis of right ventricular outflow tract reconstruction.