Kimberley G. Miles, Dan M. Dorobantu, Johannes Menno Douwes, Benjamin S. Frank, Pei-Ni Jone, Vivek Muthurangu, D. Dunbar Ivy, Hythem Nawaytou, Mark K. Friedberg
Cincinnati Children’s Hospital and University of Cincinnati College of Medicine. Bristol Royal Hospital for Children and Heart Institute. Beatrix Children’s Hospital, University Medical Center Groningen and University of Groningen. University of Colorado Denver Anschutz Medical Center and Children’s Hospital Colorado. Lurie Children’s Hospital and Northwestern University Feinberg School of Medicine. University College London. University of California, San Francisco. Hospital for Sick Children and University of Toronto.
United States, United Kingdon, Netherlands and Canada
Circulation Cardiovascular Imaging
Circ Cardiovasc Imaging 2026;
DOI: 10.1161/CIRCIMAGING.125.019264
Abstract
Right ventricular (RV) failure is a principal determinant of morbidity and mortality in children with pulmonary hypertension, making accurate RV assessment a cornerstone of risk stratification and long-term management. Noninvasive imaging plays a central role in this evaluation; however, commonly used modalities, including 2- and 3-dimensional echocardiography and cardiac magnetic resonance imaging, each have distinct advantages and limitations in the pediatric population. Consequently, an integrated, multimodal imaging strategy is required. This review provides a contemporary, critical appraisal of the existing evidence and key knowledge gaps related to noninvasive multimodality imaging of the RV in pediatric pulmonary hypertension. The discussion is structured around fundamental aspects of RV physiology, including chamber size and mass, systolic function, diastolic function and stiffness, RV-left ventricle interactions, ventriculoarterial coupling, and exercise assessment. Echocardiography and cardiac magnetic resonance imaging are presented in parallel to highlight their complementary roles within a multimodality framework. Current prognostic thresholds in pediatric pulmonary hypertension imaging are largely supported by level of evidence C, underscoring persistent gaps that limit the development of definitive clinical recommendations and a unified approach. We propose a roadmap to guide future research efforts and collaborative initiatives among pediatric pulmonary hypertension and imaging specialists, emphasizing the role of professional networks in advancing the field.
Category
Heart Dysfunction Associated with Pulmonary Vascular Disease. Right and Left
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
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