个体化手术方案矫治儿童右室双出口的体会

目的 探讨儿童右室双出口(DORV)的外科治疗效果.方法 自1984年8月至2010年6月共手术治疗118例DORV,其中男62例,女56例,年龄10 d～12岁,平均(3.2±2.6)岁,体重3.0～22.5 kg,平均(9.6±3.7)kg.根据SIS-EACTS数据库分类:VSD型86例,TOF型22例,TGA型3例,VSD远离大动脉型7例.手术方法包括采用单纯心室内隧道修补21例,心室内隧道修补+右室流出道扩大补片84例,其中82例自体心包扩大补片,2例行同种带瓣外管道;Switch术4例,双向格林术6例,Fontan术1例,肺动脉环缩术1例,B-T分流术1例.回顾性分析DORV的手术方法和效果.结果 住院死亡9例,病死率7.6%,主要死亡原因为低心排综合症.2004年前手术51例,死亡7例(病死率13.7%),2005年后手术67例,死亡2例(病死率2.9%).存活病例随访2个月～10年,超声心动图检查未见左室流出道梗阻,1例患儿术后2年发现右室流出道残余梗阻,再次手术疏通右室流出道,余患儿效果良好,无晚期死亡病例.结论 根据DORV患儿的解剖特点和年龄,制订个体化的手术方案,有助于提高手术成功率.

Abstract：

Objective To summarize our experience of surgical treatment for double-outlet right ventricle (DORV) in children. Methods From August 1984 to June 2010, 118 patients with DORV underwent surgical repair at this center. Among the 118 patients, 62 were males and 56 were females.Their ages ranged from 10 days to 12 years old (mean age, 3. 2 ± 2. 6). Their weight ranged from 3. 0to 22. 5 kg (mean weight, 9. 6 ± 3. 7 kg). According to the STS-EACTS international nomenclature,86 patients were DORV associated with ventricular septal defect (VSD), 22 were DORV with tetralogy of Fallot (TOF), 3 were DORV with transposition of the greater arteries (TGA), and 7 were DORV with remote VSD. Corrective surgeries included 22 interventricular repairs, 84 interventricular repairs plus right ventricular outflow tract reconstruction (pericardium was used for the reconstruction in 82 cases, and valved extracardiac conduits was used in 2 cases), 4 switch procedures, 6 bidirectional Glenn procedures, 1 Fontan procedure, 1 Pulmonary artery banding procedure, and 1 Blalock-Taussig Shunt procedure. The surgical safety and efficacy were studied by retrospectively analyzing the clinical data of the 118 cases. Results Nine patients died after surgery (7. 6%). The leading cause of death was low cardiac output syndrome. Of the 51 patients underwent corrective surgery before 2004,7 died after surgery (13. 7%). However, only 2 died in the 67 patients who had surgery after 2005(2. 9%). The patients were followed up for 2 months to 10 years. Cardiac ultrasonography didn't show any obstruction of left ventricular outflow tract, but 1 patient developed left ventricular outflow tract obstruction 2 years after surgery and received corrective surgery. No long-term death and other complications were noted. Conclusions Surgical strategy for the children with double-outlet right ventricle should be made based on patients individual anatomic anomalies.

Surgical strategy for the children with double-outlet right ventricle: our experience in 118 cases

Objective To summarize our experience of surgical treatment for double-outlet right ventricle (DORV) in children. Methods From August 1984 to June 2010, 118 patients with DORV underwent surgical repair at this center. Among the 118 patients, 62 were males and 56 were females.Their ages ranged from 10 days to 12 years old (mean age, 3. 2 ± 2. 6). Their weight ranged from 3. 0to 22. 5 kg (mean weight, 9. 6 ± 3. 7 kg). According to the STS-EACTS international nomenclature,86 patients were DORV associated with ventricular septal defect (VSD), 22 were DORV with tetralogy of Fallot (TOF), 3 were DORV with transposition of the greater arteries (TGA), and 7 were DORV with remote VSD. Corrective surgeries included 22 interventricular repairs, 84 interventricular repairs plus right ventricular outflow tract reconstruction (pericardium was used for the reconstruction in 82 cases, and valved extracardiac conduits was used in 2 cases), 4 switch procedures, 6 bidirectional Glenn procedures, 1 Fontan procedure, 1 Pulmonary artery banding procedure, and 1 Blalock-Taussig Shunt procedure. The surgical safety and efficacy were studied by retrospectively analyzing the clinical data of the 118 cases. Results Nine patients died after surgery (7. 6%). The leading cause of death was low cardiac output syndrome. Of the 51 patients underwent corrective surgery before 2004,7 died after surgery (13. 7%). However, only 2 died in the 67 patients who had surgery after 2005(2. 9%). The patients were followed up for 2 months to 10 years. Cardiac ultrasonography didn't show any obstruction of left ventricular outflow tract, but 1 patient developed left ventricular outflow tract obstruction 2 years after surgery and received corrective surgery. No long-term death and other complications were noted. Conclusions Surgical strategy for the children with double-outlet right ventricle should be made based on patients individual anatomic anomalies.