Stevens Christina G, Lipsitz Stuart, Thiagarajan Ravi R, Hames Daniel L
Department of Cardiology, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.
Harvard Medical School, Boston, MA, USA.
Pediatr Cardiol. 2025 Jul 18. doi: 10.1007/s00246-025-03961-y.
Patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) occasionally require perioperative extracorporeal membrane oxygenation support (ECMO). Outcomes and associations with survival in this cohort are poorly described. This study utilized an international database to identify the associations with mortality and cardiac non-recovery in children with ALCAPA receiving perioperative ECMO. Retrospective analysis was performed using the Extracorporeal Life Support Organization registry database for patients with ALCAPA undergoing ECMO cannulation between 2005 and 2022. Demographic information, clinical characteristics, and ECMO-related variables and complications were compared. Of 163 patients with ALCAPA analyzed, overall survival to discharge was 74%. Non-survivors had lower weight and suffered more complications on ECMO compared with survivors. In multivariable analysis, time to ECMO (OR 1.03, 95% CI 1.01-1.05), pre-ECMO pH (OR 0.02, 95% CI 0.01-0.67), length of ECMO run (OR 1.01, 95% CI 1.00-1.01), and presence of mechanical (OR 6.33, 95% CI 1.81-22.1) and neurologic complications (OR 7.6, 95% CI 2.1-27.9) had increased odds for poor outcome. Factors associated with mortality and cardiac non-recovery for patients with ALCAPA on ECMO include pre-ECMO variables and accrued complications on ECMO. Thoughtful consideration of timing of ECMO deployment and careful ECMO management to prevent complications are necessary to improve outcomes in this population.
左冠状动脉起源于肺动脉(ALCAPA)的患者偶尔需要围手术期体外膜肺氧合支持(ECMO)。该队列中的预后情况以及与生存的关联鲜有描述。本研究利用一个国际数据库来确定接受围手术期ECMO的ALCAPA患儿的死亡及心脏功能未恢复的相关因素。使用体外生命支持组织登记数据库对2005年至2022年间接受ECMO插管的ALCAPA患者进行回顾性分析。比较了人口统计学信息、临床特征、与ECMO相关的变量及并发症。在分析的163例ALCAPA患者中,出院时的总体生存率为74%。与幸存者相比,非幸存者体重更低,在ECMO期间出现更多并发症。在多变量分析中,开始ECMO的时间(比值比1.03,95%置信区间1.01 - 1.05)、ECMO前pH值(比值比0.02,95%置信区间0.01 - 0.67)、ECMO运行时间(比值比1.01,95%置信区间1.00 - 1.01)以及存在机械性(比值比6.33,95%置信区间1.81 - 22.1)和神经系统并发症(比值比7.6,95%置信区间2.1 - 27.9)会增加不良预后的几率。对于接受ECMO的ALCAPA患者,与死亡及心脏功能未恢复相关的因素包括ECMO前变量以及ECMO期间出现的并发症。为改善该人群的预后,需要慎重考虑ECMO启动的时机并进行仔细的ECMO管理以预防并发症。