Related Experiment Video
Updated: Jul 30, 2025

Author Spotlight: Establishment and Confirmation of a Postnatal Right Ventricular Volume Overload Mouse Model
Published on: June 9, 2023
Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements
Meindina G Haarman1, Iris Coenraad1, Quint A J Hagdorn1
1Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, 9700 RB Groningen, The Netherlands.
Insights
Cardiac MRI measures of right ventricular function and remodeling predict survival in pediatric idiopathic/heritable pulmonary arterial hypertension (PAH). These findings can aid risk stratification for children with PAH.
Area of Science:
- Cardiology
- Pediatric Imaging
- Pulmonary Hypertension
Background:
- Pulmonary arterial hypertension (PAH) significantly impacts right ventricular (RV) function and remodeling, crucial for patient outcomes.
- Risk stratification in pediatric PAH requires reliable noninvasive prognosticators.
- The prognostic utility of RV characteristics from cardiac magnetic resonance (CMR) in pediatric PAH is underexplored.
Purpose of the Study:
- To identify CMR-derived morphometric and functional RV characteristics that predict outcomes in children with PAH.
- To assess the prognostic value of specific RV parameters in pediatric patients with idiopathic/heritable PAH (IPAH/HPAH) and PAH associated with congenital heart disease (PAH-CHD).
Main Methods:
- Retrospective analysis of CMR data from 38 children with severe PAH (IPAH/HPAH or PAH-CHD).
- Evaluated RV ejection fraction (RVEF), indexed RV mass (RVMi), RV/LV mass ratio (RVM/LVM-ratio), and left ventricular eccentricity index (LVEI).
- Correlated these parameters with transplant-free survival.
Main Results:
- In children with IPAH/HPAH, RVEF, RVMi, RVM/LVM-ratio, and LVEI were significantly correlated with transplant-free survival.
- These correlations were not observed in the PAH-CHD subgroup.
- Patients presented with severe PAH, indicated by WHO Functional Class, elevated NT-proBNP, and high PAP/PVRi.
Conclusions:
- CMR-derived RV function and remodeling metrics (LVEI, RVMi, RVM/LVM-ratio, RVEF) are prognostic for transplant-free survival in pediatric IPAH/HPAH.
- These imaging parameters hold potential for inclusion in risk stratification scores for pediatric PAH management.
Abstract:
Pulmonary arterial hypertension (PAH) is associated with increased right ventricular (RV) afterload, affecting RV remodeling and RV performance, a major determinant of outcome in PAH-patients. In children with PAH, treatment strategy is guided by risk stratification where noninvasive prognosticators are highly needed. The prognostic value of RV characteristics derived by cardiac magnetic resonance (CMR) has been scarcely studied in pediatric PAH. We aimed to identify CMR-derived morphometric and functional RV characteristics prognostic for outcome in children with PAH. From the Dutch National cohort, thirty-eight children with either idiopathic/heritable PAH (IPAH/HPAH) or PAH associated with congenital heart disease (PAH-CHD), who underwent CMR, were included (median (interquartile range) [IQR] age 13.0 years (10.8-15.0), 66% females). Patients had severe PAH, characterized by their World Health Organization Functional Class, increased N-terminal pro-B-type natriuretic peptide and high pulmonary arterial pressure and pulmonary vascular resistance index at time of CMR. RV-ejection fraction (RVEF), indexed RV-mass (RVMi), the ratio between RV and LV mass (RVM/LVM-ratio) and left ventricular eccentricity index (LVEI) all correlated with transplant-free survival from time of CMR. These correlations could not be confirmed in the PAH-CHD group. This study shows that CMR-derived measures reflecting RV function and remodeling (LVEI, RVMi, RVM/LVM-ratio, RVEF) predict transplant-free survival in children with IPAH/HPAH and may be included in risk stratification scores in pediatric PAH.
More Related Videos
Related Concept Videos
Aortic Regurgitation II: Clinical Features and Diagnostic Tests
Mitral Stenosis II: Clinical features and Diagnostic Tests
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Mitral Stenosis I: Introduction

