John T. Wren Jr., Rachael M. Hyland, Patrick J. McNamara
University of Iowa Healthcare.
United States
Journal of Pediatrics
J Pediatr 2026;
DOI: 10.1016/j.jpeds.2026.115044
Abstract
Objectives: To define clinically the initial (<24 hour) hemodynamic phenotypes (no/mild pulmonary hypertension [PH], pre-capillary PH, and post-capillary PH) in neonates with congenital diaphragmatic hernia (CDH) by echocardiography, and to assess their relationship with clinical outcomes.
Study design: This was a retrospective, single-center, observational study that included all neonates with CDH with an echocardiogram in the first 24 hours from 2018-2025. Phenotypes were categorized via an a priori-defined algorithm, characterized by clinical and echocardiography indices and were evaluated for association with mortality, need for extracorporeal life support (ECLS), and key clinical outcomes.
Results: Despite bidirectional atrial and/or ductal shunts in every echocardiogram (n=28), phenotype identification was feasible with 2 (7%) no/mild PH, 18 (64%) pre-capillary PH, and 8 (29%) post-capillary PH phenotype. There was no association between phenotype and mortality or ECLS; however, the post-capillary phenotype was associated with earlier mortality, decreased surgical repair, and mortality when exposed to inhaled nitric oxide.
Conclusions: An algorithmic approach that includes early echocardiography can reliably identify hemodynamic phenotypes even in the presence of bidirectional shunts. Phenotypes may have distinct clinical trajectories in neonates with CDH.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
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: No