Stephanie M. Tsoi, Philip T. Levy, Steven H. Abman, Nidhy P. Varghese
Benioff Children’s Hospital and University of California, San Francisco. Harvard Medical School and Boston Children’s Hospital. University of Colorado Anschutz Medical School and Children’s Hospital Colorado. Baylor College of Medicine and Texas Children’s Hospital.
United States
Journal of Perinatology
J Perinatol 2026;
DOI: 10.1038/s41372-026-02704-y
Abstract
The earliest clinical sign of pulmonary vascular disease in neonates is persistent pulmonary hypertension of the newborn (PPHN), traditionally classified as World Symposium on Pulmonary Hypertension (WSPH) Group 1. Typical PPHN is defined by the delayed transition from intra- to extra- uterine life with failure of pulmonary artery pressures to fall after delivery. This results in sustained elevation of pulmonary vascular resistance and contributes to hypoxemic respiratory failure (HRF). However, early HRF can also arise from atypical PPHN phenotypes that may persist beyond the expected resolution of typical PPHN and are often classified within WSPH Groups 2, 3 and 5. In addition, a history of PPHN can be associated with the childhood and adulthood PH. In this perspective, we highlight the diverse etiologies contributing to the PPHN phenotype, with a focus on Group 3 disease, and propose a physiology-based framework to classify PPHN and delineate disease trajectories.
Category
Class I. Persistent Pulmonary Hypertension of the Newborn
Class III. Pulmonary Hypertension Associated with Lung Disease
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Class III. Pulmonary Hypertension Associated with Developmental Diseases of the Lung
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes