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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 anatomic features of patients with visceral ectopic syndrome and the clinical efficacy of Fontan’s surgery. Methods The data of 81 inpatients with visceral syndrome from September 2008 to September 2013 in Shanghai Children’s Medical Center Cardiac Center were retrospectively analyzed. There were 49 males and 32 females. The median operative age was 3.79 (2.07 ~ 13.02) years old and the weight was 14.8 (10.0 ~ 36.0) kg. The preoperative oxygen saturation was 81% (63% -97%). Results There were 70 cases of right atrium heterogeneous and 11 cases of left atrial isomerism. There were 16 cases of right atrium, 50 cases of single atrium, 44 cases of single ventricle, 40 cases of conotruncal malformation, 72 cases of pulmonary stenosis, 8 cases of pulmonary atresia, 54 cases of bilateral superior vena cava; common atrioventricular valve in 61 cases. Fifty-seven patients underwent Fontan operation and 24 patients underwent Fontan operation during the first phase. The surgical methods included 17 cases of atrial / external catheterization, 48 cases of external catheterization, 14 cases of endocardial tunneling and 2 cases of pulmonary artery pull-down. Postoperative early death 15 cases. Postoperative complications included 15 cases of low cardiac output syndrome, 35 cases of hepatic insufficiency, 50 cases of peritoneal dialysis and 28 cases of arrhythmia. The oxygen saturation before discharge was 89% (78% -98 %). Outpatients were followed up for 2 to 72 months, followed up a total of 64 cases, 2 patients due to thrombosis external pipe replacement. Conclusions Patients with visceral ectopic syndrome are mostly isomerism in the right atrium, which is quite different from the reports in western countries. Fontan surgery is still the mainstay of treatment for this disease. Strict admission of indications is the key to improving clinical outcomes.