功能性单心室的分期外科手术疗效及术后死亡危险因素分析

Effect of multistage surgery in patients with functional single ventricle and risk factors of postoperative death

目的 探讨功能性单心室的分期外科手术疗效及术后死亡的危险因素.方法 采用回顾性研究方法,纳入广州市妇女儿童医疗中心心脏中心2008年1月至2017年12月连续收治并接受单心室分期姑息手术的功能性单心室患儿289例.患儿年龄中位数为10.0 (6.0,35.4)个月,男性198例,女性91例.分析其临床资料,并对患儿进行随访.采用Kaplan-Meier法计算术后生存率,患儿术后死亡的危险因素分析采用多因素Cox回归分析.结果 接受第一期姑息手术患儿79例,接受第二期Glenn手术患儿232例,接受第三期Fontan手术患儿162例.术后死亡39例,包括第一期姑息手术后死亡21例(早期13例,晚期8例),第二期Glenn手术后死亡10例(早期和晚期各5例),第三期Fontan手术死亡8例(早期6例,晚期2例).Kaplan-Meier法生存分析显示,功能性单心室患儿术后总体1、5和10年生存率分别为90.2% (95%CI 86.7%~ 93.7%)、85.9%(95%CI 81.8%~ 90.0%)和84.6%(95%CI 79.7%~ 89.5%);第一期姑息手术后1、5和10年生存率分别为74.4% (95%CI 64.8%~ 84.0%)、73.0%(95%CI 63.2% ~ 82.8%)和73.0%(95%CI 63.2%~82.8%);第二期Glenn手术后1、5和10年生存率分别为97.8% (95%CI 95.8% ~ 99.7%)、95.2% (95%CI 92.3%~98.1%)和95.2% (95%CI 92.3%~98.1%);第三期Fontan手术后1和5年生存率分别为95.6% (95%CI 92.5%~ 98.7%)和93.7%(95 %CI88.8%~98.6%).多因素Cox回归分析显示,合并完全性肺静脉异位连接(HR=5.47,95%CI2.71~11.04,P<0.001)、中重度房室瓣反流(HR=2.52,95%CI 1.32~ 4.79,P=0.005)、体循环流出道梗阻(HR=3.47,95%CI 1.30~ 9.29,P=0.013)和需要行第一期姑息手术(HR=3.12,95%CI 1.59~ 6.15,P=0.001)是患儿术后死亡的危险因素.结论 功能性单心室的分期外科手术可有效提高生存率,改善患儿远期预后.合并完全性肺静脉异位连接、中重度房室瓣反流、体循环流出道梗阻和需要行第一期姑息手术是功能性单心室患儿术后死亡的危险因素.

Objective To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death.Methods The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed.The study included 289 patients.The age was 10.0 (6.0,35.4) months,and there were 198 male and 91 female patients.The patients were followed up at outpatient clinic.Survival rates were calculated with Kaplan-Meier.Multivariate Cox regression analysis was made to determine the risk factors of postoperative death.Results Seventy-nine patients required the first stage palliation surgery,232 patients received the Glenn shunt surgery,and 162 patients completed the Fontan procedure.Overall,postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases,late stage 8 cases),10 after the Glenn shunt surgery (early stage 5 cases,late stage 5 cases),and 8 following the Fontan procedure (early stage 6 cases,late stage 2 cases).Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%),85.9% (95%CI 81.8%-90.0%),and 84.6% (95%CI 79.7%-89.5%) at 1 year,5 years and 10 years post operation.Survival rate was 74.4% (95%CI 64.8%-84.0%),73.0%(95%CI 63.2%-82.8%),and 73.0% (95%CI 63.2%-82.8%) at 1 year,5 years,and 10 years post the first stage palliation surgery,97.8% (95%CI 95.8%-99.7%),95.2% (95%CI 92.3%-98.1%),and 95.2%(95%CI 92.3%-98.1%) at 1 year,5 years,and 10 years post Glenn shunt surgery,95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery.Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47,95%CI 2.71-11.04,P<0.001),atrioventricular valve regurgitation more than moderate (HR=2.52,95%CI 1.32-4.79,P=0.005),systemic ventricular outflow tract obstruction (HR=3.47,95%CI 1.30-9.29,P=0.013),and required the first palliation surgery (HR=3.12,95%CI 1.59-6.15,P=0.001) were risk factors of postoperative death.Conclusions The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis.Total anomalous pulmonary venous connection,atrioventricular valve regurgitation more than moderate,systemic ventricular outflow tract obstruction,and required the first palliation surgery are risk factors of postoperative death in these patients.