KU Leuven, Faculty of Medicine, Leuven, Belgium.
UZ Leuven, Pediatric Cardiology, Leuven, Belgium; KU Leuven, Department of Cardiovascular Sciences, Leuven, Belgium.
Int J Cardiol. 2024 Nov 15;415:132476. doi: 10.1016/j.ijcard.2024.132476. Epub 2024 Aug 22.
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.
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.
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).
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.
肺动脉闭锁、室间隔缺损伴粗大侧支（PA-VSD-MAPCAs）是一种先天性紫绀型心脏病，预后较差。由于其复杂且高度可变的解剖结构，最佳治疗方案尚不清楚。我们的目的是：（1）根据基础原始解剖结构和治疗策略，研究 PA-VSD-MAPCAs 患者的生存率；（2）评估手术与非手术治疗后严重发育不良的 native pulmonary arteries（NPAs）对患者预期寿命的影响。
我们查阅了在鲁汶大学医院治疗和随访的 169 例 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 Kaplan-Meier 生存曲线，并使用 Log Rank 检验进行比较。
PA-VSD-MAPCAs 患者的总体平均生存率为 38.5 年（95%-CI：33.1-43.9）。完全心内修复的患者平均生存时间最长，为 43.8 年（95%-CI：38.1-49.6），明显优于其他组（p<0.001）。NPAs 正常且发育良好的患者具有更长的平均无事件生存时间（p=0.047）。最后，对于接受手术治疗的患者，NPAs 发育不良或缺失的患者的生存率更差。在未进行完全修复的情况下，体肺分流术或单腔化术对预后的影响有限（p=0.167）。
接受完全心内修复且/或伴有发育良好的 native pulmonary arteries 的 PA-VSD-MAPCAs 患者预后最佳。我们分析的数据表明，不完全手术修复的生存率与非手术治疗相当。
