单心室合并完全性肺静脉异位引流的外科治疗-中国循环杂志.PDF

中国循环杂志 2017 年 1 月 第 32 卷 第 1 期（总第 223 期）Chinese Circulation Journal，January，2017，Vol. 32 No.1（Serial No.223） 77 临床研究 单心室合并完全性肺静脉异位引流的外科治疗 陈伟丹，陈欣欣，王武军 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 3.3~49.5 kg，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。 关键词 心脏缺损，先天性；心脏外科手术 Surgical Treatment of Single Ventricle With Total Anomalous Pulmonary Venous Connection in Relevant Patients CHEN Wei-dan, CHEN Xin-xin, WANG Wu-jun. Department of Thoracic Cardiovascular Surgery, Southern Medical University Affiliated Nanfang Hospital, Guangzhou (510515), Guangdong, China. Corresponding Author: WANG Wu-jun, Email: wang_wujun@126.com Abstract 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.