双心室矫治完全型房室间隔缺损合并右心室双出口或法洛四联症

Biventricle repair of atrioventricular septal ddefect associated with tetralogy of Fallot or double-outlet right ventricle

目的：总结双心室矫治完全型房室间隔缺损合并右心室双出口或法洛四联症（CAVSD-DORV/TOF）的外科经验.方法：全部患者均按常规体外循环方法中低温（28℃～32℃）下手术.11例采用右心房-右心室径路,2例单纯右心房径路.应用逗号状补片修补VSD,其中双片法10例,单片法3例.5例采用单纯补片扩大右心室流出道（RVOT）,4例行跨环补片,2例行右心室-肺动脉连接术（Ras-telli）,1例单纯扩大主肺动脉,1例仅行肺动脉瓣交界切开.结果：2例死亡,均为CAVSD-DORV,VSD远离大动脉,合并镜面右位心.11例存活患者出院前超声心动图检查示左、右心室流出道无狭窄,房室瓣成形满意.10例患者随访3～77个月,心功能均为NYHAⅠ级或Ⅱ级,无再次手术发生.结论：双心室矫治CAVSD-ROVR/TOF近期及中期效果满意,可作为首选手术策略.采用右心房-右心室径路,应用逗号状补片双片法修补室缺以及精细的房室瓣成形是提高手术成功率,减少并发症的关键.

Objective：To summarize our ten years surgical experience of biventricle repair of atrioventricular septal defect associated with tetralogy of Fallot or double-outlet right ventricle（CAVSD-DORV/TOF）.Methods：13 patients were performed with the aid of cardiopulmonary bypass with moderate systemic hypothermia and cold crystalloid cardioplegia.11 cases were through the transatrial-transventricle operative approach,2 were only transatrial approach.The two-patch technique was usued to correct the atrioventricular septal defect in 10 cases,3 were single-patch.Right ventricular outflow tract reconstruction：5 cases with simple patch-expansion of RVOT,4 cases transannular patch,2 cases right ventricle to pulmonary connection （Rastelli）,1 case of simple expansion of the main pulmonary artery,and 1 was only simple pulmonary valvotomy.Results：2 cases were dead,all were the CAVSD-DORV,VSD was noncommited,combined with mirror dextrocardia.The 11 survived patients show a good surgical result by echocardiography examination before hospital discharge.The left and right ventricular outflow tract stenosis relief and atrioventricular valve forming is satisfied.10 patients were followed up from 3 to 77 months,the cardiac function of NYHA was class Ⅰ or class Ⅱ,no patient need reoperation.Conclusion：Biventricle repair of CAVSD-DORV/TOF is our current surgical strategy with a good intermediate survival and moderate follow-up result.Transatrial-transventricle surgical approach,two-patch technique and comma shape patch,accurately valvuloplasty is the key for good surgical outcome.