完全性大动脉转位合并左室流出道狭窄患儿行不同心室内修复手术的回顾性队列研究  

Prognosis of the complete transposition of great arteries with left ventricular outflow tract obstruction after intraventricular repair

作　　者：邢云超 李守军[1] 闫军[1] 王旭[1] 晏馥霞[1] 亦桐 蒋显超 马志岭 王强[1] XING Yunchao;LI Shoujun;YAN Jun;WANG Xu;YAN Fuxia;Yl Tong;JIANG Xianchao;MA Zhiling;WANG Qiang(Pediatric Cardiac Surgical Centre, Cardiovascular Institute and Fuwai Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100037,P.R.China)

机构地区：[1]中国医学科学院北京协和医学院阜外医院心外科

出　　处：《中国胸心血管外科临床杂志》2019年第6期534-538,共5页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：首都特色临床医学应用发展基金(2016-2-4034)

摘　　要：目的探索行两种不同的心室内修复术的患儿临床特征和预后水平及其术后不良事件和相关危险因素。方法回顾性分析2012年1月1日至2017年1月1日我院24例行心室内修复术的完全性大动脉转位(complete transposition of the great arteries,TGA)/左室流出道狭窄(left ventricular outflow tract obstruction,LVOTO)(TGA/LVOTO)患儿的临床资料。按手术方式将患者分为改良REV组和Rastelli组。REV组13例,男9例、女4例,中位月龄25.2(6,72)个月;Rastelli组11例,男10例、女1例,中位月龄47.9(14,144)个月。结果 REV组和Rastelli组患儿手术时的年龄(P=0.041)、肺动脉瓣环内径的标准Z值(P=0.002)和左室流出道峰值压差(P=0.004)、多期手术比例(P=0.005)差异有统计学意义。平均随访时间为17.3个月,随访期间有1例患儿发生了术后早期死亡,术后早期再干预2例,7例患儿发生了右室流出道狭窄(right ventricular outflow tract obstruction,RVOTO),且行Rastelli手术(P=0.028),室间隔缺损内径较大(P=0.029)的患儿术后更易发生RVOTO。结论作为TGA/LVOTO的传统手术方式,患儿术后早期死亡率很低,早期再干预也较少,改良REV术后易出现新构建肺动脉瓣反流,行Rastelli手术和室间隔缺损较大的患儿易出现术后的RVOTO,但在平均17个月的随访中因术后环肺静脉隔离(PVI)和RVOTO造成的远期再干预率很低,故总体预后较好。Objective To compare the clinical characteristics and prognosis of patients who received two different intraventricular repair. Methods We retrospectively analyzed the clinical data of 24 complete transposition of the great arteries(TGA)/left ventricular outflow tract obstruction(LVOTO) patients who all received intraventricular repair. The patients were allocated into two groups including a REV group and a Rastelli group. There were 13 patients with 9 males and 4 females at median age of 25.2(6, 72) months in the REV group. There were 11 patients with 10 males and 1 female at median age of 47.9(14, 144) months in the Rastelli group. Results The age at operation(P=0.041),pulmonary valve Z value(P=0.002), and LVOT gradient(P=0.004), rate of multiphase operation between the REV group and the Rastelli group was statistically different. The mean follow-up time was 17.3 months. And during the follow-up, 1 patient had early mortality, 2 patients had early reintervention, 7 patients had postoperative RVOTO, and received Rastelli and larger VSD inner diameter were associated with postoperative RVOTO. Conclusion As the traditional surgery for TGA/LVOTO patients, the intraventricular repair has a low early mortality and low early reintervention. Modified REV is associated with postoperative peripheral pulmonary vein isolation(PVIS). Patients who received Rastelli operation and with larger VSD inner diameter are more likely to have postoperative RVOTO, but the reintervention for PVI and RVOTO during follow up is very low.

关 键 词：完全性大动脉转位 左室流出道狭窄 心室内修复术 预后分析 

分 类 号：R726.5[医药卫生—儿科]