摘要目的 研究全腔肺吻合拆除治疗婴幼儿Fontan术后急性心衰的效果.方法 2013年11月至2017年12月,共447例患儿行Fontan手术,回顾性分析其中14例术后出现急性心功能衰竭(心衰)并行Fontan拆除手术的患儿资料.男9例,女5例,年龄(4.3±1.4)岁;身高(98.9±8.1)cm;体质量(14.3±2.7)kg.一期Fontan手术2例;分期Fontan术12例,双向腔肺吻合术(Glenn)后(2.6±1.1)年行Fontan术.5例为外管道Fontan,9例为心内管道,11例采用管道开窗技术.14例均拆除将Fontan循环,至改良双向腔肺吻合状态:4例Fontan术后无法脱离体外循环即刻拆除,10例术后入监护室出现心衰,内科治疗无明显好转,遂拆除Fontan循环,对14例患儿行死亡相关因素分析.结果 Fontan拆除术后4例死亡,出院生存比例71.4％.2例拆除后安装ECMO装置,其中1例死亡.生存患儿ICU停留(17.0±11.2)天,平均住院(33.8±19.4)天.死亡相关单因素分析显示Fontan手术至拆除时间与拆除后死亡相关.结论 Fontan拆除术可以有效治疗婴幼儿Fontan术后急性心衰,Fontan术后患儿出现药物治疗无效的急性心衰需尽早行Fontan拆除术.

abstractsTo analyze the outcomes of early Fontan failure after Fontan takedown. Methods A retrospective study of early Fontan Failure(EFF) children undergoing Fontan takedown from November 2013 to December 2017 was performed. Fontan takedown was defined as takedown back to an intermediate circulation, superior cavopulmonary connection. Fontan takedown was performed in 14 patients. There were 9 boys and 5 girls. Children were on average aged(4. 3 ± 1. 4) years when they had Fontan procedure. The mean weight was(14. 3 ±2. 7)kg. Operative procedure was extra-cardiac Fontan in 5 children, 9 had intra-cardiac Fontan. Fenestration was used in 11/14 patients. The outcomes were summarized with statistics, and risk factors for mortality after Fontan takedown were identified. Results The mortality after Fontan takedown was 4/ 14(28. 6％). In two patients(14. 3％), ECMO was followed after takedown, one of them died after two days. The time of ICU stay and hospital stay was relatively long(17. 0 ±11. 2)days and(33. 8 ±19. 4)days. The interval time between the Fontan procedure and the takedown operation is the risk fact after Fontan takedown. Conclusion Fontan takedown can be used as an effective management for the early Fontan failure, still with a high risk of mortality. Early diagnosis and takedown is recommended for EFF.