背景:肺动脉闭锁,心室依赖性缺损,主要主动脉肺副动脉(PA-VSD-MAPCAs)是一种先天性紫癜性心脏病,预后不良。由于其复杂和高度可变的解剖结构,最佳治疗方案尚不明确。我们的目的是(1)根据基础原始解剖和治疗策略调查PA-VSD-MAPCAs患者的生存,和(2)评估手术与非手术治疗后有或没有严重原生肺动脉发育不良的患者之间的预期寿命。
方法:访问了在鲁汶大学医院接受治疗和随访的169名PA-VSD-MAPCAs患者的前瞻性数据库。根据治疗策略将患者分为三组。绘制了Kaplan-Meier存活曲线,和LogRank测试用于比较。
结果:PA-VSD-MAPCAs患者的总平均生存期为38.5年(95%-CI:33.1-43.9)。与其他组相比,完成心内修复的患者的最长平均生存期为43.8年(95%-CI:38.1-49.6)(p<0.001)。在正常的患者中发现了更长的平均无事件生存时间,发达的NPAs(p=0.047)。最后,NPAs发育不良或缺乏NPAs的患者在遵循手术方法时生存率较差.在没有完全修复的情况下,体肺分流术放置或单域化对预后的影响有限(p=0.167)。
结论:接受完全心内修复和/或天然肺动脉发育良好的PA-VSD-MAPCAs患者预后最好。我们分析的数据表明,不完全的手术修复导致的生存率与非手术方法相当。
BACKGROUND: Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-
VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-
VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment.
METHODS: A prospectively established database of 169 PA-
VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison.
RESULTS: The overall mean survival for patients with PA-
VSD-MAPCAs was 38.5 years (95%-CI: 33.1-43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1-49.6) versus the other groups (p < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167).
CONCLUSIONS: Patients with PA-
VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach.