Abstract：
BACKGROUND: Pulmonary 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.
METHODS: A 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.
RESULTS: Survival 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.
CONCLUSIONS: Long-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.
METHODS: A 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.
RESULTS: Survival 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.
CONCLUSIONS: Long-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.
摘要：
背景：伴有室间隔缺损且无主肺侧支动脉（MAPCAs）的肺动脉闭锁是一种罕见的先天性心脏病。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结果.
方法：使用相关关键词（肺动脉闭锁，法洛四联症,导管,右心室-肺动脉）集中于1990年至今的研究，并以英文发表。大多数肺动脉闭锁-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，考虑MAPCAs患者的比例，以及没有MAPCAs的患者的任何亚组分析。
结果：10年生存率约为80％，并且随着完全修复和更大的肺动脉而得到改善。一些研究发现遗传综合征和心外异常会影响生存率，而其他人没有。已显示不完全修复与更差的存活率相关。独立于初始管理战略，没有MAPCA的肺动脉闭锁和室间隔缺损患者需要对右心室流出道进行重复干预。肺动脉发育不全已被证明是再干预的危险因素，导管植入年龄越小，导管耐久性下降。
结论：无MAPCAs的肺动脉闭锁室间隔缺损患者的长期结局有所改善，完全修复和足够的肺动脉有利于生存。长期结果包括再干预，基于导管的和手术的，主要位于右心室流出道。
方法：使用相关关键词（肺动脉闭锁，法洛四联症,导管,右心室-肺动脉）集中于1990年至今的研究，并以英文发表。大多数肺动脉闭锁-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，考虑MAPCAs患者的比例，以及没有MAPCAs的患者的任何亚组分析。
结果：10年生存率约为80％，并且随着完全修复和更大的肺动脉而得到改善。一些研究发现遗传综合征和心外异常会影响生存率，而其他人没有。已显示不完全修复与更差的存活率相关。独立于初始管理战略，没有MAPCA的肺动脉闭锁和室间隔缺损患者需要对右心室流出道进行重复干预。肺动脉发育不全已被证明是再干预的危险因素，导管植入年龄越小，导管耐久性下降。
结论：无MAPCAs的肺动脉闭锁室间隔缺损患者的长期结局有所改善，完全修复和足够的肺动脉有利于生存。长期结果包括再干预，基于导管的和手术的，主要位于右心室流出道。
