Long-Term Outcomes of Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries.

IF 1.5 4区医学 Q3 SURGERY

ANZ Journal of Surgery Pub Date : 2025-06-02 DOI:10.1111/ans.70193

Samuel J Macalister, Igor E Konstantinov, Tyson A Fricke, Robert G Weintraub, Christian P Brizard, Edward Buratto

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引用次数: 0

Abstract

Background: While complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is achieved with low surgical mortality, this condition poses ongoing mid- and late-term mortality. The long-term data for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries at a single institution are presented in this paper.

Methods: We reviewed the outcomes for 100 patients with PA/VSD/MAPCAs who had surgery between January 1987 and July 2018. Two patients were excluded who had palliative surgery not intended to reach a complete repair.

Results: Of the 98 patients who were suitable candidates for complete repair, 13% (13/98) had primary complete repair and 64% (63/98) had staged complete repair. Estimated survival at 1, 5, 10, and 20 years of age was 93%, 87%, 81%, and 76%, respectively. The median age of those patients alive at follow-up was 15 (interquartile range 9-21) years. Freedom from reintervention after staged complete repair was 58% at 5 years after complete repair. Ligating or embolizing MAPCAs was associated with a decreased hazard of death in the staged complete repair group (hazard ratio 0.6, p-value 0.04).

Conclusions: A strategy of staged complete repair can be used to achieve complete repair and stable long-term survival for patients with PA/VSD/MAPCAs.

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肺动脉闭锁合并室间隔缺损及主动脉-肺侧支的远期疗效观察。

背景：虽然完全性肺动脉闭锁合并室间隔缺损和主动脉-肺动脉副支（PA/VSD/MAPCAs）的手术死亡率较低，但这种情况会导致持续的中期和晚期死亡率。本文介绍了在同一医院对合并室间隔缺损和主肺动脉侧枝的肺动脉闭锁进行修复的长期资料。方法：我们回顾了1987年1月至2018年7月期间接受手术的100例PA/VSD/MAPCAs患者的结果。两名患者被排除在外，他们接受了姑息性手术，但没有达到完全修复的目的。结果：98例适合完全修复的患者中，13%（13/98）进行了一期完全修复，64%（63/98）进行了分期完全修复。1岁、5岁、10岁和20岁的估计生存率分别为93%、87%、81%和76%。随访时存活患者的中位年龄为15岁（四分位数范围9-21岁）。完全修复后5年，分期完全修复后再干预率为58%。在分阶段完全修复组中，结扎或栓塞MAPCAs与死亡风险降低相关（风险比0.6，p值0.04）。结论：分期完全修复策略可使PA/VSD/MAPCAs患者实现完全修复和稳定的长期生存。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

ANZ Journal of Surgery 医学-外科

CiteScore

2.50

自引率

11.80%

发文量

720

审稿时长

2 months

期刊介绍： ANZ Journal of Surgery is published by Wiley on behalf of the Royal Australasian College of Surgeons to provide a medium for the publication of peer-reviewed original contributions related to clinical practice and/or research in all fields of surgery and related disciplines. It also provides a programme of continuing education for surgeons. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.