Section of Neonatology, Department of Pediatrics, University of Colorado Anschutz School of Medicine and Children's Hospital Colorado, Aurora, CO, United States; Pediatric Heart Lung Center, Department of Pediatrics, University of Colorado Anschutz School of Medicine and Children's Hospital Colorado, Aurora, CO, United States.
Pediatric Heart Lung Center, Department of Pediatrics, University of Colorado Anschutz School of Medicine and Children's Hospital Colorado, Aurora, CO, United States; Section of Pulmonary Medicine, Department of Pediatrics, University of Colorado Anschutz School of Medicine and Children's Hospital Colorado, Aurora, CO, United States.
Semin Fetal Neonatal Med. 2022 Aug;27(4):101367. doi: 10.1016/j.siny.2022.101367. Epub 2022 Jun 3.
The management of acute hypoxemic respiratory failure (AHRF) in newborns continues to be a clinical challenge with elevated risk for significant morbidities and mortality, especially when accompanied with persistent pulmonary hypertension of the newborn (PPHN). PPHN is a syndrome characterized by marked hypoxemia secondary to extrapulmonary right-to-left shunting across the ductus arteriosus and/or foramen ovale with high pulmonary artery pressure and increased pulmonary vascular resistance (PVR). After optimizing respiratory support, cardiac performance and systemic hemodynamics, targeting persistent elevations in PVR with inhaled nitric oxide (iNO) therapy has improved outcomes of neonates with PPHN physiology. Despite aggressive cardiopulmonary management, a significant proportion of patients have an inadequate response to iNO therapy, prompting consideration for additional pulmonary vasodilator therapy. This article reviews the pathophysiology and management of PPHN in term newborns with AHRF while highlighting both animal and human data to inform a physiologic approach to the use of PH-targeted therapies.
急性低氧性呼吸衰竭（AHRF）的管理仍然是一个临床挑战，存在较高的严重发病率和死亡率风险，尤其是伴有新生儿持续性肺动脉高压（PPHN）时。PPHN 是一种以动脉导管和/或卵圆孔发生显著的肺外右向左分流为特征的综合征，导致严重低氧血症，同时伴有肺动脉高压和肺血管阻力（PVR）增加。在优化呼吸支持、心功能和全身血液动力学后，针对持续性 PVR 采用吸入一氧化氮（iNO）治疗，改善了具有 PPHN 病理生理学的新生儿的结局。尽管进行了积极的心肺管理，但仍有相当一部分患者对 iNO 治疗反应不佳，促使考虑使用其他肺血管扩张剂治疗。本文回顾了伴有 AHRF 的足月新生儿 PPHN 的病理生理学和管理，同时强调了动物和人体数据，为 PH 靶向治疗的使用提供了一种基于生理学的方法。
