Chen Xinxin.Current therapeutic status and recent advances for Congenitally corrected transposition of the great arteries[J].Journal of Clinical Pediatric Surgery,2021,20(06):506-511.[doi:10.12260/lcxewkzz.2021.06.002]
纠正型大动脉转位的治疗进展
- Title:
- Current therapeutic status and recent advances for Congenitally corrected transposition of the great arteries
- Keywords:
- Transposition of Great Vessels/SU; Transposition of Great Vessels/DI; Transposition of Great Vessels/PP; Heart Defects; Congenital/SU; Treatment Outcome
- 分类号:
- R541.1;R493.28;R322.11
- 摘要:
- 纠正型大动脉转位是一种较为罕见的复杂先天性心脏病，常伴有室间隔缺损、肺动脉狭窄或闭锁、三尖瓣Ebstein样畸形以及中位心等。其病理解剖的基本特征是心房心室连接不一致，心室大动脉连接不一致。1957年开展了生理性纠正手术，二十世纪八十年代末期开展了解剖性纠正手术，即双调转手术，包括心房调转加动脉调转手术和心房调转加心室调转手术，均取得良好效果，且中远期随访结果也令人满意。二十一世纪后，又相继开展了11/2心室的解剖性纠正手术，即Hemi-Mustard、双向Glenn及Rastelli手术。同时对于纠正型大动脉转位合并室间隔缺损及肺动脉狭窄的部分患者采用了Fontan手术，亦取得了较好的中远期效果。对于室间隔完整的纠正型大动脉转位患者来说，采用肺动脉环缩术及房间隔造口术，可以有效锻炼退化的形态学左心室，达到施行解剖性纠正手术的目的。生理性纠正手术因其具有不良的中远期结果而逐渐被弃用，解剖性纠正手术因恢复了正常的解剖生理而成为主流的手术方式。在一些选择性的解剖性纠正手术患者中，Fontan手术也取得了令人满意的中远期结果。对于我国从事先心病外科治疗的医生来说，解剖性纠正手术需要一定数量的复杂性先心病外科手术经验作为支撑，也依赖于治疗团队的综合实力和哲学思想。
- Abstract:
- As a complex and rare congenital heart disease,congenitally corrected transposition of the great arteries (ccTGA) is frequently complicated with ventricular septal defect,pulmonary artery stenosis or atresia,tricuspid Ebstein-like malformation and mesocardia.The basic characteristics of its pathological anatomy include atrioventricular discordance and arterioventricular discordance.Physiological correction was performed in 1957 while anatomical correction,namely double reversal surgery,implemented in late 1980s,including atrial switch and arterial switch with satisfactory surgical outcomes.The medium/long-term follow-ups were also satisfactory.After the 21st century,anatomic correction of one and a half ventricles,including Hemi-Mustard,bidirectional Glenn and Rastelli.At the same time,some ccTGA children with ventricular septal defect and pulmonary artery stenosis underwent Fontan operation with decent medium/long-term outcomes.For ccTGA children with intact ventricular septum,pulmonary artery banding and atrial septostomy could effectively retrain the degenerate morphological left ventricle and achieve ultimate anatomical correction.Physiological correction has been gradually abandoned due to its poor medium/long-term outcomes.Anatomical correction has become a major option since it restores normal anatomy and physiology.Fontan procedure has also yielded satisfactory medium/long-term outcomes in patients with high risks for anatomic correction.For surgical treatment of congenital heart disease,anatomical correction not only requires a certain amount of complex surgical experience,but also depends upon the comprehensive strength and philosophy of treatment team.
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备注/Memo
收稿日期:2021-04-13;。
基金项目:广州市高水平临床重点专科
通讯作者:陈欣欣,Email:zingerchen@163.com