三种姑息手术方法治疗肺动脉闭锁伴室间隔缺损的对比研究  

A comparative study of three palliative surgical approaches for pulmonary atresia with ventricular septal defect

作　　者：宋治莹 郑景浩[1] 何晓敏 罗凯 孙琦[1] 陈会文 祝忠群[1] 张浩[1] 徐志伟[1] 刘锦纷[1] SONG Zhiying;ZHENG Jinghao;HE Xiaomin;LUO Kai;SUN Qi;CHEN Huiwen;ZHU Zhongqun;ZHANG Hao;XU Zhiwei;LIU Jinfen(Department of Cardiothoracic Surgery,Shanghai Children’s Medical Center,Shanghai Jiao Tong University School of Medicine,Shanghai,200127,P.R.China)

机构地区：[1]上海交通大学医学院附属、上海儿童医学中心心胸外科,上海200127

出　　处：《中国胸心血管外科临床杂志》2023年第2期266-272,共7页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：上海市科学技术委员会资助项目(19411963700)。

摘　　要：目的比较和探讨改良B-T分流术、中央分流术和右心室肺动脉(right ventricle-pulmonary artery,RV-PA)连接术治疗肺动脉闭锁伴室间隔缺损(pulmonary atresia with ventricular septal defect,PA/VSD)的疗效差异。方法2014年9月—2019年8月在上海儿童医学中心共124例PA/VSD患儿接受了一期姑息手术,其中男63例、女61例,年龄7 d~15岁。根据手术方式将患儿分为三组:改良B-T分流组55例,中央分流组22例和RV-PA连接组47例。比较三组患儿的临床资料。结果总体一期姑息术后早期死亡9例,死亡率为7.3%。平均随访(26.5±20.3)个月,5例失访,5例远期死亡,远期存活105例,术后1年、5年生存率均为89.7%。改良B-T分流组、中央分流组和RV-PA连接组Nakata指数每月增加5.2(–0.2,12.3)mm^(2)/m^(2)、9.2(0.1,23.6)mm^(2)/m^(2)、6.3(1.8,23.3)mm^(2)/m^(2),三组之间差异无统计学意义(P>0.05);术后1年生存率分别为85.3%、78.4%、95.2%,5年生存率(中央分流组为4年生存率)分别为85.3%、58.8%、95.2%,三组之间差异有统计学意义(P<0.05);根治率分别为36.5%、19.0%和67.4%,三组之间差异有统计学意义(P<0.001)。结论三种姑息手术方法都可以有效促进肺血管发育。但相比于体肺分流术,RV-PA连接术围术期死亡率低,后期可以获得更高的根治率,有利于长期预后。Objective To compare and investigate the efficacy and differences of modified B-T shunt,central shunt and right ventricle-pulmonary artery(RV-PA)connection in the treatment of pulmonary atresia with ventricular septal defect(PA/VSD).Methods A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children’s Medical Center from September 2014 to August 2019,including 63 males and 61 females,aged 7 days to 15years.They were divided into in a modified B-T shunt group(55 patients),a central shunt group(22 patients)and a RV-PA connection group(47 patients).The clinical data of these children were retrospectively analyzed.Results There were9 early deaths after palliation,with an early mortality rate of 7.3%.The mean follow-up time was 26.5±20.3 months,with 5patients lost to follow-up,5 deaths during the follow-up period,and 105 survivors.The 1-year and 5-year survival rates were both 89.7%.The monthly increased Nakata index was 5.2(–0.2,12.3)mm^(2)/m^(2),9.2(0.1,23.6)mm^(2)/m^(2),6.3(1.8,23.3)mm^(2)/m^(2)in the modified B-T shunt group,the central shunt group,and the RV-PA connection group,respectively with no statistical difference among the three groups.The 1-year survival rate was 85.3%,78.4%,95.2%,and the 5-year(4-year in the central shunt group)survival rate was 85.3%,58.8%,95.2%in the three groups,respectively,with a statistical difference among them(P<0.05).The complete repair rate was 36.5%,19.0%and 67.4%in the three groups,respectively,with a statistical difference among the three groups(P<0.001).Conclusion All these three palliative surgical approaches can effectively promote pulmonary vascular development.But compared with systemic-pulmonary shunt,RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage,which is beneficial for long-term prognosis.

关 键 词：肺动脉闭锁伴室间隔缺损 体肺分流手术 右心室肺动脉连接术 

分 类 号：R726.5[医药卫生—儿科]