Stephens Elizabeth H, Dearani Joseph A, Miranda William R, Anderson Jason H
Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
World J Pediatr Congenit Heart Surg. 2025 Mar;16(2):232-236. doi: 10.1177/21501351241254034. Epub 2024 Jul 23.
BackgroundPulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population.MethodsA review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs.ResultsSurvival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit.ConclusionsLong-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.
背景
无主要体肺侧支动脉（MAPCAs）的室间隔缺损合并肺动脉闭锁是一种罕见的先天性心脏病形式。随着越来越多的先天性心脏病患者活到成年，本文的目的是回顾这一特定人群的长期治疗结果。
方法
使用相关关键词（肺动脉闭锁、法洛四联症、管道、右心室 - 肺动脉）对PubMed数据库进行检索，重点关注1990年至今以英文发表的研究。大多数关于肺动脉闭锁合并室间隔缺损患者的研究包括有和没有MAPCAs的患者。分析包括对整个队列的检查、对有MAPCAs患者比例的考量以及对没有MAPCAs患者的任何亚组分析。
结果
十年生存率约为80%，通过完全修复和较大的肺动脉可提高生存率。一些研究发现遗传综合征和心外异常会影响生存，而其他研究则未发现。不完全修复已被证明与较差的生存率相关。无论初始治疗策略如何，无MAPCAs的肺动脉闭锁合并室间隔缺损患者都需要对右心室流出道进行再次干预。发育不良的肺动脉已被证明是再次干预的危险因素，并且在管道植入时年龄较小会导致管道耐久性降低。
结论
无MAPCAs的肺动脉闭锁合并室间隔缺损患者的长期治疗结果有所改善，完全修复和足够的肺动脉有利于生存。长期治疗结果包括再次干预，包括基于导管的和手术的，主要针对右心室流出道。
