摘要
目的:总结单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:入选2008-09至2016-03期间,我院手术治疗的单心室合并TAPVC患儿25例,年龄5天~14岁,体重3.3~49.5kg,心上型TAPVC 22例,心下型TAPVC 1例,混合型TAPVC 2例;合并完全性房室间隔缺损17例,三尖瓣闭锁2例,二尖瓣闭锁2例;中度以上房室瓣反流4例,肺动脉闭锁5例,肺静脉梗阻9例。结果:院内早期死亡5例,死亡率20%(5/25),死亡原因均为低心排血量综合征。存活20例患儿随访1~65个月,7例再次手术,其中5例完成二期改良Fontan术,1例因重度房室瓣反流行房室瓣置换术,另1例因肺静脉梗阻及重度房室瓣反流行肺静脉狭窄矫治+房室瓣成形术;余13例患儿未再次手术。20例出院患者随访期间3例死亡,死亡原因分别为低心排血量综合征、蛋白丢失性肠病和神经系统损伤。余存活患儿一般情况好,未发现残余肺静脉梗阻。结论:单心室合并TAPVC常合并右心房异构,心内畸形复杂,手术治疗仍具有较高死亡率。
Objective: To summarize surgical result of single ventricle with total anomalous pulmonary venous connection (TAPVC) in relevant patients. Methods: A total of 25 patients of single ventricle with TAPVC treated in our hospital from 2008-09 to 2016-03 were summarized. The patients were between 5 days to 14 years of age with body weight at (3.3-49.5) kg. There were 22 patients with supracardiac type TAPVC, 1 with infracardiac type TAPVC and 2 with mixed type TAPVC; in addition,17 cases having complete atrio-ventricular canal defect, 2 having tricuspid atresia, 4 having more than moderate atrio-ventricular valve regurgitation, 5 having pulmonary atresia and 9having pulmonary venous obstruction. Results: There were 5/25 (20%) patients died at the early stage for low cardiac output syndrome. Followed-up study was conducted in 20 survivals for (1-65) months and 7 patients had re-surgery including 5 with modified Fontan operation, 1 with atrio-ventricular valve replacement due to severe atrio-ventricular valve regurgitation, 1 with pulmonary venous stenosis repair + atrio-ventricular valve replacement due to pulmonary venousobstruction and severe atrio-ventricular valve regurgitation. 3 patients died during followed-up period because of low cardiac output syndrome, protein lost intestine disease and neurological injury respectively. The rest survivals had good general condition, no residualpulmonary venous obstruction was found. Conclusion: The patients of single ventricle with TAPVC were frequently associated with right atrial isomerism and complex cardiac anomaly; surgical treatment still having the high mortality.
出处
《中国循环杂志》
CSCD
北大核心
2017年第1期77-80,共4页
Chinese Circulation Journal