改良Fontan手术治疗复杂先天性心脏病

Modified Fontan Procedure for Complex Congenital Heart Diseases

目的总结Fontan术治疗儿童复杂先天性心脏病的临床经验。方法回顾性分析2008年5月至2013年12月广州市妇女儿童医疗中心62例行改良Fontan术复杂先天性心脏病患儿的临床资料,男41例、女21例,年龄1岁4个月至14岁,中位年龄4岁;体重12.5（8.9~49.5）kg。功能性单心室45例,大动脉转位合并室间隔缺损及左心室流出道梗阻6例,矫正型大动脉转位合并室间隔缺损及左心室流出道梗阻6例,右心室双出口合并重度肺动脉狭窄4例,右心室发育不良1例。前期手术包括肺动脉环缩术10例,单侧双向Glenn 37例,双侧Glenn 8例。行一期Fontan术17例,二期Fontan术45例。Fontan手术方式包括心房内侧隧道Fontan术6例,心外管道Fontan术56例;其中伴随手术包括开窗术41例,房室瓣成形术6例,肺动脉成形术3例。结果一期和二期Fontan术组各死亡2例,死亡率分别为11.8%和4.4%（P=0.299）。两组术后机械通气时间、住ICU时间、胸腔引流时间、术后住院时间及术后主要并发症差异均无统计学意义（P〉0.05）。术后随访3个月至5年,平均（2.0±0.5）年。随访期间一期Fontan术组死亡2例,二期Fontan术组无死亡。生存患儿生长发育良好,活动能力明显改善,经皮血氧饱和度〉90%;超声提示上腔、下腔吻合口均通畅,无血栓及狭窄形成,房室瓣反流无加重,肺静脉回流无梗阻,心功能分级（NYHA）Ⅰ~Ⅱ级;无心律失常、慢性渗出、蛋白丢失性肠病等并发症。结论改良Fontan术治疗儿童复杂先天性心脏病早中期效果满意。对于合并Fontan手术危险因素的患儿,分期Fontan手术可降低手术死亡率。

Objective To review our clinical experience of modified Fontan procedure for complex congenital heart disease（CHD） in children. Methods Clinical data of 62 children with complex CHD who underwent modified Fontan procedure in Guangzhou Women and Children＇s Medical Center from May 2008 to December 2013 were retrospectively analyzed. There were 41 male and 21 female patients with their median age of 4 years（range, 16 months to 14 years） and body weight of 12.5（8.9-49.5） kg. Diagnosis included functional single ventricle in 45 patients, transposition of the great arteries（TGA） with ventricular septal defect（VSD） and left ventricular outflow tract obstruction（LVOTO） in 6 patients, corrected TGA with VSD and LVOTO in 6 patients, double outlet right ventricle with severe pulmonary stenosis in 4 patients, and right ventricular dysplasia in 1 patient. Previous procedure included pulmonary artery banding in 10 patients, unilateral bidirectional Glenn shunt in 37 patients, and bilateral bidirectional Glenn shunt in 8 patients. Seventeen patients received single-stage modified Fontan procedure, and 45 patients received two-stage modified Fontan procedure. Extracardiac conduit was used in 56 patients, and lateral tunnel was used in 6 patients. Concomitant procedures included fenestration in 41 patients, atrioventricular valvuloplasty in 6 patients, and pulmonary artery angioplasty in 3 patients. Results Two patients in both single-stage and two-stage groups died postoperatively with mortality of 11.8% and 4.4% respectively（P=0.299）. Postoperative mechanical ventilation time, length of ICU stay, chestdrainage duration, postoperative hospital stay and morbidity were not statistically different between single-stage and two-stage group. Mean follow-up was 2.0 ± 0.5 years（range, 3 months to 5 years）. There were 2 late death in the singlestage group but no late death in the two-stage group. Growth of all survival children was good, and their exercise capacity significantly improved. Percutaneous oxygen saturation was higher than 90%. Echocardiography showed patent superior and inferior vena cava anastomosis without thrombosis, stenosis, atrioventricular valve regurgitation aggravation or pulmonary venous return obstruction. All survival patients were in New York Heart Association class Ⅰ or Ⅱ. None of the patients had arrhythmia, chronic effusion or protein losing enteropathy. Conclusion Early and mid-term results of modified Fontan procedure were satisfactory for children with complex CHD. For children with high risk factors, staged Fontan procedure can reduce surgical mortality.