右心室流出道重建术治疗复杂先天性心脏病的临床研究——单中心10年随访分析  

Clinical study of right ventricular outflow tract reconstruction for complex congenital heart disease——a single-center 10-year follow-up analysis

作　　者：罗凯 郑景浩[1] 祝忠群[1] 潘燕军 何晓敏 徐志伟[1] 刘锦纷[1] LUO Kai;ZHENG Jinghao;ZHU Zhongqun;PAN Yanjun;HE Xiaomin;XU Zhiwei;LIU Jinfen(Department of Cardiothoracic Surgery,Shanghai Children's Medical Center,Shanghai Jiaotong 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》2019年第2期125-131,共7页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：国家重点研发计划课题(2017YFC1308103)

摘　　要：目的总结分析多种右心室流出道手术治疗复杂性先天性心脏病(先心病)的临床经验。方法回顾性分析2007年1月至2017年1月我院胸外科3 138例右心系统复杂性先心病患儿的临床资料,其中男1 660例、女1 478例,手术年龄9 d～84个月,体质量2.2～28.6 kg。所有患儿均完成右心室流出道重建术,手术方法包括肺动脉补片扩大术2 335例,自体组织连接右心室289例,外管道重建右心室流出道514例。结果术后早期死亡181例(5.8%),死亡原因主要为严重低心排血量综合征,肺高压危象及右心衰竭。中远期死亡15例(术后6～27个月),死亡原因主要为心功能不全和突发猝死。术后共随访2 782例患儿,中远期随访结果:右心室流出道梗阻408例,肺动脉及分支狭窄340例,中度以上肺动脉瓣反流309例。394例患儿实施二次手术,再手术率为12.6%,术后死亡39例,死亡率为9.9%。随访过程中,92.4%患儿心功能恢复良好,心功能(NYHA)分级达到Ⅱ级及以上。结论右心室流出道覆盖范围广,解剖结构复杂多变,手术方法多样,且各具优势,中远期预后均较满意。手术方案应根据病变类型、解剖特点、临床症状以及手术条件等多因素进行个体化制定。Objective To assess the mid-and long-term outcomes of right ventricular outflow tract reconstruction for children with congenital heart disease. Methods We retrospectively analyzed the clinical data of 3 138 children with complex congenital heart disease in right heart system admitted to our hospital from January 2007 to January 2017. There were 1 660 males and 1 478 females. The age at surgery was 9 days to 84 months, and the body weight was 2.2 to 28.6 kg. Pulmonary patch enlargement was performed in 2 335 patients(1 477 patients of valve-sparing repair and 858 patients of transannular repair);autologous tissue(direct anastomosis, left auricle or pericardial conduit) was used to connect with right ventricle in 289 patients;extracardiac conduits were used for reconstruction in 514 patients.Results There were 181 in-hospital deaths with a mortality of 5.8%. The early postoperative causes of death were low cardiac output syndrome(LCOS), severe pulmonary hypertension and right heart failure. Fifteen patients died of cardiac insufficiency or sudden death during follow-up(6–27 months postoperatively). The echocardiography showed 408 patients with right ventricular outflow tract obsturction(RVOTO), 340 patients with pulmonary trunk or branches stenosis, 609 with pulmonary regurgitation(morderate or severe). 12.6%(394/3 138) of patients underwent reintervention or reoperation with 39 deaths. About 92.4% of patients exhibited an improvement of New York Heart Association(NYHA) functional class from Ⅲ or Ⅳ preoperatively to Ⅰ or Ⅱ at follow-up. Conclusion The anatomical structure of right ventricular outflow tract is complicated and various, and each operation method has different strengths and favorable outcomes. The operation should be individually designed according to pathological types, anatomical features, clinical symptoms and operation conditions.

关 键 词：复杂先天性心脏病 右心室流出道重建术 自体组织 外管道 

分 类 号：R726.5[医药卫生—儿科]