双向Glenn分流术治疗儿童复杂紫绀型先天性心脏病

The application of bi-directional Glenn shunt for treatment of 155 children with complicated cyanotic congenital heart diseases

目的总结双向Glenn(BDG)分流术在治疗儿童复杂紫绀型先天性心脏病(先心病)的经验和体会。方法 1999年1月至2004年10月,对155例具有Fontan术高危因素的复杂先心病作BDG术。高危因素包括:年龄<2岁,左和(或)右肺动脉狭窄,平均肺动脉压升高,房室瓣反流及体静脉或(和)肺静脉异位回流等。术前诊断包括单心室(53例)、右室双出口(44例)、完全性大动脉转位(9例)、纠正性大动脉转位(4例)、三尖瓣闭锁(9例)、肺动脉闭锁伴室间隔完整(11例)、肺动脉闭锁伴室间隔缺损(17 例)、上下心室(7例)和法洛四联症伴完全性房室间隔缺损(1例)。其中56例为内脏异位综合征(HS) (36．1％)。130例和25例分别在体外循环和非体外循环下手术。手术类型包括单侧BDG术(84例),双侧BDG术(52例),下腔静脉-肺动脉外管道BDG术(4例),半Fontan术(13例),右半Fontan术十左BDG 术(2例)。其他手术包括完全性肺静脉(TAPVC)(8例)和部分性肺静脉(3例)异位回流纠治术,房室瓣修复术(12例),肺动脉重建术(26例)。结果术后早期平均动脉氧饱和度由术前0．75±0．07升高至 0．86±0．05。手术早期病死率1．9％(3/155例)。结论 BDG分流术可广泛应用于小儿复杂紫绀型先心病,尤适宜于无法行双室修复术,而又存在Fontan术高危因素病儿。HS手术时,术前诊断有无TAPVC十分重要,在作BDG术同时应行TAPVC纠治术。保留肺动脉前向性血流指征为BDG术后PaO2<30 mm Hg,SaO2<0．75,如SaO2>0．85则部分关闭肺动脉干。就医较晚的10岁以上病儿,行下腔静脉-肺动脉外管道BDG术不失为有效治疗方法。

Objective To review the experiences of BDG shunt in treatment of children with complicated cyanotic congenital heart diseases （CHD）. Methods From Jan. 1999 through Oct. 2004, one hundred and fifty five patients in whom a modified Fontsn operation was postponed because of multiple risk factors underwent a BDG shunt. The following risk factors were age ycxmger than 2 years, dysplasia of right and/or left pulmonary arteries, increased mean pulmonary artery pressure, atrioventricular valve regurgitation and anomalous systemic or/and pulmonary venous connection. The preoperative diagnosis were single ventricle in 53 patients, danble outlet of right ventricle in 44, transposition of great arteries in 9, corrected transposition of great arteries in 4, tricuspid atresia in 9, pulmonary atresia with intact ventricular septum in 11, pulmonary atresia with ventricular septal defect in 17, superior-inferior ventricles in 7 and tetralogy of Fallot with complete atrioventricular septal defect in 1. There were 56 patients with hetemtaxy syndrome （HS） （36.1%）. One hundred and thirty patients were operated upon with extracorpereal circulation and 25 patients without CPB. Eighty-four patients underwent unilateral BDG shunt, 52 bilateral BDG shunts, 4 inferior vena cava to pulmonary artery BDG shunt with extra cardiac conduit, 13 hemi-Fontsn procedure and 2 right hemi-Fontsn procedure with left BDG shunt. Concomitant procedures included correction of TAPVC （8） and PAPVC （3）, atrioventricular valve repair （12） and pulmonary artery reconstruction （26）. Results Early post-operative mean oxygen saturation increased from 0.75 ± 0.07 to 0.86 ± 0.05. The early operative mortality was 1.9% （3/155）. Conclusion The BDG shunt has been widely applied to a variety of complicated cyanotic CHD and has been found a useful interim palliation in high-risk Fontsn candidates. The preoperative diagnosis of TAPVC is very important for the operation in heterotaxy syndrome. Concomitant TAPVC correction should be performed when BDG shunt is done. The indication of keeping antegrade pulmonary blood flow is PaO2 〈 30 mmHg, SaO2 〈 0.75 postoperatively. The inferior vena cava to pulmonary artery BDG with extra cardiac conduit could be performed for the teen-aged patients.