John T. Wren, Jr., Neil Patel, Patrick J. McNamara, Patrick Sloan
University of Iowa, Royal Hospital for Children. Washington University in St. Louis.
United States and United Kingdom
Children
Children 2026; 13:
DOI: 10.3390/children13091137
Abstract
Congenital diaphragmatic hernia (CDH) is increasingly recognized as a dynamic cardiopulmonary disease in which pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction interact to shape clinical instability, therapeutic response, and outcomes. Hemodynamic phenotyping has emerged as a strategy to move beyond binary classification of pulmonary hypertension and toward physiology-directed care. Early single-center experiences suggest potential clinical utility of echocardiography-guided, phenotype-directed management; however, external validation remains limited. Further important challenges remain, including technical and institutional barriers to timely echocardiography, limitations of static single-time-point assessments, uncertainty regarding what exactly defines each phenotype, and incomplete understanding of the impact of time and therapies on phenotype presentations. In this perspective, we summarize the evolution of heart-focused care in CDH, describe current hemodynamic phenotyping approaches, examine unresolved questions in phenotype classification and implementation, and outline future research priorities needed to advance dynamic, mechanism-based precision cardiopulmonary care for infants with CDH.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
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