复杂先天性心脏病从单心室姑息转换为双心室解剖矫治的外科策略  

Surgical strategies of biventricular conversion from single-ventricle palliation for the complex congenital heart defects

作　　者：郑景浩[1] 何晓敏 潘燕军 孙琦[1] 祝忠群[1] 张浩[1] Zheng Jinghao;He Xiaomin;Pan Yanjun;Sun Qi;Zhu Zhongqun;Zhang Hao(Department of Cardiothoracic Surgery,Shanghai Children's Medical Center,Shanghai Jiao Tong University School of Medicine,Shanghai 200127,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,200127

出　　处：《中华胸心血管外科杂志》2021年第11期664-668,共5页Chinese Journal of Thoracic and Cardiovascular Surgery

基　　金：国家重点研发计划项目(2017YFC1308100);促进市级医院临床技能与临床创新能力三年行动计划(SHDC2020CR4093);上海市卫生健康委员会卫生行业临床研究专项面上项目(202040337)。

摘　　要：目的探讨及总结部分复杂先天性心脏病(先心病)拟行单心室姑息而最终转换为双心室解剖纠治的外科策略。方法2017年4月至2019年12月,14例原计划拟行单心室矫治的复杂先心病患儿最终转换为双心室解剖矫治,依据左、右心室发育及解剖特性分为2组:左右心室对称组(9例),均表现心内畸形复杂,但左、右心室发育对称,包括室间隔缺损远离型右心室双出口4例、大动脉转位伴室间隔缺损伴肺动脉狭窄2例、矫正型大动脉转位伴右心室双出口伴主动脉弓离断1例、右心室双出口伴肺动脉瓣下室间隔缺损伴房室连接不一致1例和肺动脉闭锁伴室间隔缺损1例;左、右心室不对称组(5例),均为功能性单心室,包括心脾综合征3例、心室不平衡型完全性房室间隔缺损2例。对两组的手术时间、术后并发症、死亡、心脏功能及再手术情况等进行比较分析。结果左右心室对称组接受双心室解剖矫治等待期(56.4±38.9)个月,术后死亡1例,早期并发症包括心律失常2例、胸腔积液3例和肺部感染2例。左右心室不对称组接受双心室解剖矫治等待期(20.4±15.8)个月,不对称侧的心室舒张期末容积(33.6±2.1)ml/m^(2),术后无死亡,早期并发症包括低心排血量综合征2例、心律失常1例和肾功能衰竭1例。两组手术时间、气管插管和ICU时间比较无统计学差异。术后随访4个月~3年,无院外死亡病例,左右心室对称组1例因上腔静脉与右心耳吻合口梗阻而再手术,左右心室不对称组1例因Ⅲ度房室传导阻滞而置入心内膜永久性起搏器,全组心功能NYHA分级均为Ⅰ-Ⅱ级。结论不论左右心室发育良好或不对称的复杂先心病在计划拟行单心室矫治术前,通过术前的综合评估均有助于发现适宜病例,根据其解剖特性采取相应的双心室转换外科策略可获得较为满意的临床疗效,但中远期结果仍需进一步随访。Objective To explore and summarize the surgical strategies of biventricular conversion from single-ventricle palliation for the complex congenital heart defects in this study.Methods From April 2017 to December 2019,a total of 14 cases with complex complex congenital heart defects who had planned to undergo single-ventricle palliation were finally accepted biventricular conversion.According to the development and anatomical characteristics of the ventricles,they were divided into two groups:ventricular well development group(9 cases),all of which showed complex intracardiac defects,and the ventricles well developed,including 4 cases of double outlet right ventricle with remote ventricular septal defect,2 cases of transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction,one case of congenitally corrected transposition of the great arteries combined with double outlet right ventricle and interrupted aortic arch,one case of double outlet right ventricle with subpulmonary ventricular septal defect and atrioventricular connection inconsistency,one case of pulmonary atresia with ventricular septal defect.Ventricular unbalanced development group(5 cases),all of which were functional single ventricle,including 3 cases of heterotaxy and 2 cases of unbalanced complete atrioventricular septal defect.The operation time,postoperative complications,death,cardiac function and reoperation of the two groups were compared.Results There was one early death,with a mortality rate of 7.1%.In the ventricular well development group,the average waiting time for biventricular conversion was(56.4±38.9)months.One case died after the operation.The early complications included 2 case of arrhythmias,3 cases of pleural effusions and 2 cases of pulmonary infections.In the ventricular unbalanced development group,the average waiting time for biventricular conversion was(20.4±15.8)months,and the average of left ventricular end-diastolic volume was(33.6±2.1)ml/m^(2).The early complica

关 键 词：复杂先天性心脏病 外科治疗 双心室转换 单心室矫治 

分 类 号：R726.5[医药卫生—儿科]