Current State of RV Multimodality Imaging in Pediatric Pulmonary Hypertension: Current Evidence, Knowledge Gaps, and Future Research Directions

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.

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Publication Details

Journal
Circulation Cardiovascular Imaging
Published
2026-02-19
DOI
https://doi.org/10.1161/circimaging.125.019264
Primary Topic
Pulmonary Hypertension Research and Treatments
Type
article
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article

Current State of RV Multimodality Imaging in Pediatric Pulmonary Hypertension: Current Evidence, Knowledge Gaps, and Future Research Directions

Hythem Nawaytou, Johannes M. Douwes, Benjamin S. Frank, P A Jone et al.
Circulation Cardiovascular Imaging
Pulmonary Hypertension Research and Treatments
article

Current State of RV Multimodality Imaging in Pediatric Pulmonary Hypertension: Current Evidence, Knowledge Gaps, and Future Research Directions

Hythem Nawaytou, Johannes M. Douwes, Benjamin S. Frank, P A Jone, Dan Dorobanțu, Muthurangu Vivek, M. Friedberg, D. Dunbar Ivy, Kimberley G. Miles
article en

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.

Circulation Cardiovascular ImagingVol. 19(3)
Northwestern University (US), Cincinnati Children's Hospital Medical Center (US), University Medical Center Groningen (NL), University of California, San Francisco (US), Cardiovascular Institute of the South (US), Hospital for Sick Children (CA), Bristol Royal Hospital for Children (GB), Children's Hospital Colorado (US), Beatrix Kinderziekenhuis (NL), Robert H. Lurie Comprehensive Cancer Center of Northwestern University, University College London (GB), University of Cincinnati Medical Center (US), University of Colorado Denver (US)
Openalex Percentile: Top 50%
Pulmonary Hypertension Research and Treatments
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