内脏异位综合征心血管畸形的解剖特征和Fontan手术的疗效观察

Features of Cardiacvascular Abnormalities and Clinical Results in Patients with Fontan Operation for Heterotaxy Syndrome

目的总结内脏异位综合征患者的解剖特征及Fontan手术的临床疗效。方法回顾性分析上海儿童医学中心心脏中心2008年9月至2013年9月收治的81例内脏异位综合征患者住院病例资料。其中男49例、女32例,中位手术年龄3.79(2.07~13.02)岁,体重14.8(10.0~36.0)kg,术前血氧饱和度81%(63%~97%)。结果右心房异构70例,左心房异构11例;合并右位心16例,单心房50例;单心室44例,圆锥动脉干畸形40例,肺动脉瓣狭窄72例,肺动脉闭锁8例,双侧上腔静脉54例;共同房室瓣61例。行分期Fontan手术57例,一期Fontan手术24例;手术方法包括心房内/外管道法17例、外管道法48例、心内侧隧道法14例和自体肺动脉下拉法2例;术后早期死亡15例。术后并发症包括:15例发生低心排血量综合征,35例发生肝功能不全,50例行腹膜透析术,28例发生心律失常;出院前血氧饱和度89%(78%~98%)。门诊随访2~72个月,随访总数64例,2例患者因血栓形成行外管道置换术。结论内脏异位综合征患者以右心房异构居多,与西方国家文献报道差别较大。Fontan手术仍然是该疾病的主要治疗方式,严格的适应证准入是提高临床疗效的关键。

Objective To summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome.Method We retrospectively analyzed the medical records of 81 patients who underwent the Fontan operation with heterotaxy syndrome between September 2008 and September 2013.There were 49 male and 32 female patients at age of 3.79(range 2.07-13.02) years with preoperative room air saturation of 81%(range 63%-97%)and weight of 14.8(10.0-36.0) kg.Results There were 70 patients in the right atrial isomerism group and left in 11 patients.Dextrocardia was seen in 16 patients,and single atrium in 50 patients.Single ventricle was found in 44 patients,conotruncal defects in 40 patients,pulmonary stenosis in 72 patients,pulmonray atresia in 8 patients,common atrioventricular valve in 61 patients,and bilateral superior vena cava in 54 patients.The staged Fontan procedure was applied in 57 patients and one stage in 24 patients.Operation strategies were included intra/extracardiac conduit(n=17),extracardiac conduit(n=48),lateral tunnel(n=14) and direct cavopulmonray connection(n=2).There were 15 early deaths.Postoperative complications included low cardiac output in 15 patients,hepatic insufficiency in 35 patients,renal insufficiency in55 patients required peritoneal dialysis and arrhythmia in 28 patients.The room air saturation was 89%(range 78%-98%)before discharge and time of follow-up was from 2 months to 6 years in 64 patients.Thromboembolic events were found in 2 patients who had accomplished conduit replacement operation.Conclusion Compared with reported literatures in western countries,heteraotaxia patients are mostly with right atrial isomerism.Fontan palliation is still the main treatment option and strict indication was needed for satisfactory clinical results.