Nemeh Christopher, Schmoke Nicholas, Patten William, Clark Eunice, Wu Yeu S, Wang Pengchen, Kurlansky Paul, Middlesworth William, Cheung Eva W, Rosenzweig Erika B
Department of Surgery, Division of Pediatric Surgery Columbia University Vagelos College of Physicians and Surgeons/NewYork-Presbyterian Morgan Stanley Children's Hospital New York New York USA.
Division of Pediatric Cardiology, Department of Pediatrics Columbia University Vagelos College of Physicians and Surgeons/NewYork-Presbyterian Morgan Stanley Children's Hospital New York New York USA.
Pulm Circ. 2024 Oct 25;14(4):e12442. doi: 10.1002/pul2.12442. eCollection 2024 Oct.
Pediatric pulmonary arterial hypertension (PAH) can present with a wide spectrum of disease severity. Pulmonary hypertension (PH) crises can lead to acute decompensation requiring extracorporeal membrane oxygenation (ECMO) support, including extracorporeal cardiopulmonary resuscitation (eCPR). We evaluated outcomes for pediatric PH patients requiring ECMO. A single-institution retrospective review of pediatric PAH patients with World Symposium on PH (WSPH) groups 1 and 3 requiring ECMO cannulation from 2010 through 2022 ( = 20) was performed. Primary outcome was survival to hospital discharge. Secondary outcomes were survival to decannulation and 1-year survival. Of 20 ECMO patients, 16 (80%) survived to decannulation and 8 (40%) survived to discharge and 1 year follow up. Of three patients who had two ECMO runs; none survived. There were five patients who had eCPR for the first run; one survived to discharge. The univariate logistic regression model showed that venovenous ECMO was associated with better survival to hospital discharge than venoarterial ECMO, (OR: 0.12, 95% CI: 0.01-0.86, = 0.046). PH medications (administered before, during, or after ECMO) were not associated with survival to discharge. For children with decompensated PAH requiring ECMO, mortality rate is high, and management is challenging. While VA ECMO is the main configuration for decompensated PH, VV ECMO could be considered if there is adequate ventricular function, presence of a systemic to pulmonary shunt, or an intercurrent treatable illness to improve survival to discharge. A multidisciplinary approach with requisite expertise should be utilized on a case-by-case basis until more reliable data is available to predict outcomes.
小儿肺动脉高压(PAH)的疾病严重程度范围广泛。肺动脉高压(PH)危象可导致急性失代偿,需要体外膜肺氧合(ECMO)支持,包括体外心肺复苏(eCPR)。我们评估了需要ECMO的小儿PH患者的预后。对2010年至2022年期间因PH世界研讨会(WSPH)第1组和第3组疾病需要进行ECMO插管的小儿PAH患者进行了单机构回顾性研究(n = 20)。主要结局是存活至出院。次要结局是存活至拔管和1年生存率。在20例接受ECMO治疗的患者中,16例(80%)存活至拔管,8例(40%)存活至出院并接受1年随访。在3例接受两次ECMO治疗的患者中,无一人存活。有5例患者首次接受eCPR治疗,1例存活至出院。单因素逻辑回归模型显示,与静脉动脉ECMO相比,静脉静脉ECMO与更好的存活至出院相关(OR:0.12,95%CI:0.01 - 0.86,P = 0.046)。PH药物(在ECMO之前、期间或之后使用)与存活至出院无关。对于需要ECMO的失代偿性PAH儿童,死亡率很高,管理具有挑战性。虽然VA ECMO是失代偿性PH的主要配置,但如果心室功能良好、存在体肺分流或存在可治疗的并发疾病,可考虑使用VV ECMO以提高存活至出院的几率。在获得更可靠的数据来预测结局之前,应根据具体情况采用具有必要专业知识的多学科方法。