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室间隔缺损合并肺动脉闭锁：治疗方法、结果、预后因素及现状

Ventricular septal defect with pulmonary atresia: approaches, results, prognosticators and current status.

作者信息

Garg Ansh, Sharma Rajesh

机构信息

Department of Cardiac Surgery, Alder Hey Children's Hospital, East Prescot Road, Liverpool, Merseyside L14 3AB UK.

Department of Congenital and Pediatric Cardiac Surgery, Indraprastha Apollo Hospital, New Delhi, India.

出版信息

Indian J Thorac Cardiovasc Surg. 2022 Jan;38(1):28-37. doi: 10.1007/s12055-020-01133-6. Epub 2021 Jun 26.

DOI:10.1007/s12055-020-01133-6
PMID:34898873
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8630160/
Abstract

Ventricular septal defect with pulmonary atresia and major aortopulmonary collateral arteries is a complex congenital cardiac anomaly with a wide spectrum of anatomical variations. Akin to the same, the management options are also very diverse ranging from aggressive single-stage repair with unifocalisation to surgical palliation and/or staged repair and also heart transplant. There is no consensus on the best management option. This review aims at highlighting the various surgical options and proposing a management pathway suited for the subcontinent patients.

摘要

室间隔缺损合并肺动脉闭锁及大量体肺侧支动脉是一种复杂的先天性心脏畸形，具有广泛的解剖变异。与之类似，其治疗选择也非常多样，从积极的单期矫正加单灶化到手术姑息治疗和/或分期矫正，甚至心脏移植。对于最佳治疗选择尚无共识。本综述旨在强调各种手术选择，并提出一条适合该次大陆患者的治疗路径。

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[Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries].[室间隔缺损合并主-肺动脉侧支动脉的肺动脉闭锁]
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VSD PA MAPCAS presenting late with hypoplastic pulmonary vasculature. Reply to letter to Editor: "Lesson learnt about right ventricle to pulmonary artery shunt for older children and young adults with ventricular septal defect, pulmonary atresia, and hypoplastic pulmonary arteries".室间隔缺损、肺动脉闭锁、肺血管发育不良伴肺动脉发育不全的迟发性表现。致编辑的信的回复：“关于室间隔缺损、肺动脉闭锁和肺动脉发育不全的大龄儿童及年轻成人右心室至肺动脉分流术的经验教训”
Indian J Thorac Cardiovasc Surg. 2022 Sep;38(5):574. doi: 10.1007/s12055-022-01383-6. Epub 2022 Jun 14.

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J Thorac Cardiovasc Surg. 2018 Sep;156(3):1194-1204. doi: 10.1016/j.jtcvs.2018.03.153. Epub 2018 Apr 12.
2
Approaches to Pulmonary Atresia With Major Aortopulmonary Collateral Arteries.伴有主要体肺侧支动脉的肺动脉闭锁的治疗方法
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2018 Mar;21:64-74. doi: 10.1053/j.pcsu.2017.11.001.
3
Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.术前 CT 血管造影显示，在伴有室间隔缺损和主要体肺侧支血管的肺动脉闭锁中，高肺静脉指数和存在固有肺动脉具有显著的生存优势。
Eur J Cardiothorac Surg. 2017 Aug 1;52(2):225-232. doi: 10.1093/ejcts/ezx064.
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Circ Cardiovasc Interv. 2017 Apr;10(4). doi: 10.1161/CIRCINTERVENTIONS.116.004952.
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