右心室-肺动脉连接术姑息性治疗肺动脉闭锁合并室间隔缺损患儿的单中心回顾性研究  

Right ventricular-pulmonary artery connection for palliative treatment of pulmonary atresia with ventricular septal defect in children:A single-center retrospective study

作　　者：张帅 马坚锐 邱海龙 闫新建[1,2] 谢稳 任秋实 余觉愍 陈天钰 张勇 李晓华 刘付蓉[1,2] 温树生 庄建 高强[1,2] 岑坚正 ZHANG Shuai;MA Jianrui;QIU Hailong;YAN Xinjian;XIE Wen;REN Qiushi;YU Juemin;CHEN Tianyu;ZHANG Yong;LI Xiaohua;LIU Furong;WEN Shusheng;ZHUANG Jian;GAO Qiang;CEN Jianzheng(Department of Cardiovascular Surgery,Guangdong Cardiovascular Institute,Guangdong Provincial People’s Hospital,Guangdong Academy of Medical Sciences,Guangzhou,510080,P.R.China;Guangdong Provincial Key Laboratory of South China Structural Heart Disease,Guangzhou,510080,P.R.China)

机构地区：[1]广东省人民医院(广东省医学科学院)广东省心血管病研究所心外科,广州510080 [2]广东省华南结构性心脏病重点实验室,广州510080

出　　处：《中国胸心血管外科临床杂志》2025年第3期366-371,共6页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：广东省科技计划项目(2018B090944002,2020B1111170011)。

摘　　要：目的比较肺动脉闭锁合并室间隔缺损(pulmonary atresia with ventricular septal defect,PA/VSD)患者初次行补片扩大和心包卷右心室-肺动脉连接术的优劣。方法纳入广东省人民医院2010—2020年确诊PA/VSD且初次行右心室-肺动脉连接手术的患者。按照右心室-肺动脉连接的方式将患者分为两组:全心包卷右心室-肺动脉连接组(心包卷组)和心包卷补片扩大右心室-肺动脉连接组(补片扩大组)。比较两组临床数据、影像数据。结果共纳入患者51例,其中男31例、女20例,中位年龄12.57(4.57,49.67)个月。心包卷组19例,中位年龄17.17(7.33,49.67)个月;补片扩大组32例,中位年龄8.58(3.57,52.72)个月。补片扩大右心室-肺动脉连接与心包卷右心室-肺动脉连接都能明显促进肺血管的发育,手术前后肺动脉直径及McGoon指数、Nakata指数均明显增长(P<0.001)。心包卷组需要更长的体外循环时间(P<0.001)。二次手术完成根治率可达74.51%,其中补片扩大组最终完成根治术26例(26/32,81.25%),心包卷组最终完成根治术12例(12/19,63.16%)。两组患者远期根治率、死亡率差异无统计学意义(P>0.05)。结论对于PA/VSD患者,初次选用补片扩大或者心包卷右心室-肺动脉连接作为最初的姑息治疗策略都能够很好地促进肺血管的发育,并为下一次的根治手术奠定良好的基础。但是,补片扩大右心室-肺动脉连接相较于心包卷右心室-肺动脉连接操作更简单,保留部分固有肺动脉发育潜能,可作为优选术式。Objective To compare the benefits and drawbacks of primary patch expansion versus pericardial tube right ventricular-pulmonary artery connection in patients diagnosed with pulmonary atresia with ventricular septal defect(PA/VSD).Methods A retrospective study was conducted on patients diagnosed with PA/VSD who underwent primary right ventricular-pulmonary artery connection surgery at our center between 2010 and 2020.Patients were categorized into two groups based on the type of right ventricular-pulmonary artery connection:a pericardial tube group and a patch expansion group.Clinical data and imaging findings were compared between the two groups.Results A total of 51 patients were included in the study,comprising 31 males and 20 females,with a median age of 12.57(4.57,49.67)months.The pericardial tube group included 19 patients with a median age of 17.17(7.33,49.67)months,while the patch expansion group consisted of 32 patients with a median age of 8.58(3.57,52.72)months.In both groups,the diameter of pulmonary artery,McGoon index,and Nakata index significantly increased after treatment(P<0.001).However,the pericardial tube group exhibited a longer extracorporeal circulation time(P<0.001).The reoperation rate was notably high,with 74.51%of patients requiring further surgical intervention,including 26(81.25%)patients in the patch expansion group and 12(63.16%)patients in the pericardial tube group.No statistical differences were observed in longterm cure rates or mortality between the two groups(P>0.005).Conclusion In patients with PA/VSD,both patch expansion and pericardial tube right ventricular-pulmonary artery connection serve as effective initial palliative treatment strategies that promote pulmonary vessel development and provide a favorable foundation for subsequent radical operations.However,compared to the pericardial tube approach,the patch expansion technique is simpler to perform and preserves some intrinsic potential for pulmonary artery development,making it the preferred procedure.

关 键 词：肺动脉闭锁 室间隔缺损 先天性心脏病 右心室-肺动脉连接 补片扩大 心包卷 

分 类 号：R726.5[医药卫生—儿科]