双向 Glenn 术治疗婴幼儿复杂先天性心脏病  

Outcomes of bidirectional Glenn shunt for complicated cyanotic congenital heart disease

作　　者：邹明晖 王燕飞 崔虎军 夏园生 杨盛春 马力 陈伟丹 崔彦芹 陈欣欣 

机构地区：[1]广州市妇女儿童医疗中心心脏中心,510623

出　　处：《中华小儿外科杂志》2014年第8期595-598,共4页Chinese Journal of Pediatric Surgery

基　　金：国家自然科学基金（81100233）

摘　　要：目的：总结双向 Glenn 术治疗复杂先天性心脏病的临床经验。方法2008年5月至2012年12月,我科对103例具有 Fontan 术高危因素的复杂紫绀型先心病患儿行双向 Glenn 术。术前诊断：功能性单心室61例,大动脉转位12例；矫正型大动脉转位15例,右心室双出口5例,Ebstein畸形4例,肺脉闭锁合并室间隔缺损2例,右室发育不良4例。50例在体外循环下手术,53例在非体外循环下手术。手术方式包括：单侧双向 Glenn 术83例,双侧双向 Glenn 术20例,Glenn 同期行肺动脉成形术18例,房间隔切除术15例,肺动脉环缩术12例,完全性肺静脉异位引流矫治术6例,房室瓣整形术6例、房室瓣置换术2例。结果手术死亡2例,病死率1.9%。术毕平均肺动脉压力（mPAP）（16.74±2.95）mmHg。出院日不吸氧静息氧饱和度由术前（68±13）%升高至（84±6）%。术后随访6个月至3年,1例死亡；2例因房室瓣反流加重行“房室瓣置换术”；1例因顽固性上腔静脉综合征行“双向 Glenn 拆除＋B-T 分流术”；26例完成 Fontan 术；余患儿生长发育基本正常,心功能（NYHA）I-II 级,静息 SpO2（80±11）%,无吻合口狭窄。结论双向 Glenn 术可有效改善复杂紫绀先心病患儿氧饱和度,降低心室容量负荷,近期及中期效果满意。积极处理合并的危险因素,可显著提高双向 Glenn 手术效果。Objective To review the current outcomes of bidirectional Glenn （BDG）shunt in the treatment of children with complicated cyanotic congenital heart disease.Methods A total of 103 consecutive patients in high-risk Fontan procedure underwent Glenn shunt at our center from May 2008 to December 2012.The diagnoses included single ventricle （n = 61 ）,transposition of great arteries （TGA,n= 12）,corrected transposition of great arteries （c-TGA,n = 1 5 ）,double outlet of right ventricle （DORV,n = 5 ）,Ebstein anomaly （n =4）,pulmonary atresia with intact ventricular septum （PA/VSD,n = 2 ）and right ventricular hypoplasia （n = 4 ）.There were 1 7 patients with heterotaxy syndrome. A total of 83 patients underwent unilateral operations and the remainder bilaterally.Among them,the procedure was performed with cardiopulmonary bypass （CPB）（n=50） and without （n = 56）.Concomitant procedures included pulmonary artery reconstruction （n = 1 8 ）, atrial septectomy （n = 1 5 ）, pulmonary artery banding （n = 12 ）, correction of total anomalous pulmonary venous connection （TAPVC, n = 6 ）, atrioventricular valve repair （n = 6 ） and atrioventricular valve replacement （n = 2）.Results Two early operative mortalities occurred with a rate of 1 .9%.The mean pulmonary artery pressure was 16.74 ± 2.95 mmHg. And the mean systematic oxygen saturation increased from 68 ± 13% to 84±6% at discharge.The mean follow-up period was 1 .5±1 .2 （0.5-3）years.During the observational period,1 patient died.The procedures included Glenn takedown plus B-T shunt （n = 1 ）and atrioventricular valve replacement （n = 2 ）. Twenty-six patients,considered optimal candidates,underwent Fontan operation.The remainders were well-palliated with an arterial oxygen saturation of 80±1 1 % at rest,cardiac function （NYHA） I-II class and no anastomotic stenosis.Conclusions Glenn shunt is an excellent temporary palliation prior to a Fontan operation for various cyanotic CHDs leading eventually to a

关 键 词：先天性心脏病 单心室 心脏外科手术 

分 类 号：R726.5[医药卫生—儿科]