复杂先天性心脏病单心室姑息术后转双心室矫治  

Biventricular conversion for complex congenital heart defects palliated towards singleventricle repair

作　　者：李守军[1] 陈求名 花中东[1] 张浩[1] 王旭[1] 晏馥霞[1] 刘晋萍[1] 高华炜[1] 马凯[1] 张森[1] 祁磊[1] 胡盛寿[1] 

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外医院小儿外科中心,北京100037

出　　处：《中国胸心血管外科临床杂志》2017年第2期138-142,共5页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金："十二五"国家科技支撑计划项目(2011BAI11B22);北京市科技计划项目(Z141107002514002)

摘　　要：目的复杂先天性心脏病因心内畸形复杂和外科技术局限等原因选择单心室矫治。然而,单心室矫治的中远期预后远差于双心室矫治。本文总结既往复杂先天性心脏病行单心室姑息术转双心室矫治的临床结果。方法回顾性分析2013年10月至2016年3月单心室姑息术后转双心室矫治的8例复杂先天性心脏病患者的临床资料,其中男5例、女3例。患儿行Glenn姑息术时中位年龄2.6(1.0~5.9)岁,转双心室矫治手术时中位年龄6.6(4.5~11.1)岁。3例完全性大动脉转位合并室间隔缺损和左室流出道狭窄,3例室间隔缺损远离型右室双出口合并肺动脉狭窄或肺动脉闭锁,1例右室双出口合并完全性房室间隔缺损和肺动脉狭窄,1例法洛四联症。结果 8例Glenn姑息术后患者完成转双心室矫治并将上腔静脉与右房重新连接,平均体外循环时间(275.6±107.1)min,平均主动脉阻断时间(165.9±63.6)min,平均住院时间(33.6±23.0)d,平均住ICU时间(20.3±21.0)d。无手术死亡。平均随访(1.4±0.7)年,无随访死亡及再手术,随访未见窦房结功能异常、肺动脉狭窄及上腔静脉-右房吻合口狭窄。所有患儿心功能明显改善,纽约心脏学会心功能分级(NYHA)均为Ⅰ~Ⅱ级。结论尽管技术相对困难,部分单心室姑息术后的患者仍可恢复双心室矫治,近中期临床结果满意,远期结果仍需进一步随访。Objective Complex congenital heart defects are sometimes treated by Fontan palliation for various reasons. However, the middle- and long-term prognosis of single-ventricle repair is worse than that of two-ventricle repair. In this study we reported the results of biventricular conversion in these challenging patients initially palliated towards single-ventricle repair. Methods Eight patients underwent biventricular repair conversion from prior bidirectional Glenn shunt palliation in our hospital between October 2013 and March 2016. The median age in bidirectional Glenn shunt was 2.6 years （range, 1.0 to 5.9 years） and in biventricular repair conversion was 6.6 years （range, 4.5 to 11.1 years）. Three patients suffered complete transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, three double outlet right ventricle and non-committed ventricular septal defect combined with pulmonary stenosis or atresia, one double outlet right ventricle combined with complete ativentricular septal defect and pulmonary stenosis and one Tetralogy of Fallot. Results Bidirectional Glenn shunt was taken down and superior vena cava was reconnected to the right atrium in all patients. Mean cardiopulmonary bypass and aortic cross-clamp time was 275.6±107.1 min and 165.9±63.6 min, respectively. Mean length of hospital stay and ICU stay were 33.6±23.0 d and 20.3±21.0 d, respectively. At a mean follow-up of 1.4±0.7 years, there was no mortality and reoperation. No patients presented with sinoatrial node dysfunction and superior vena cava anastomotic stenosis. According to the New York Heart Association （NYHA） Functional Classification, all patients were classified as I - II. Conclusion Biventricular repair conversion can be safely performed with favorable mortality and morbidity in specific patients palliated towards single-ventricle repair. Further follow-up is needed to investigate the long-term outcomes.

关 键 词：复杂先天性心脏病 Glenn术 单心室 双心室矫治 

分 类 号：R726.5[医药卫生—儿科]