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Updated: Feb 10, 2026
Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
Published on: June 27, 2025
Proximal pulmonary vascular stiffness as a prognostic factor in children with pulmonary arterial hypertension
Richard M Friesen1,2, Michal Schäfer1,3, D Dunbar Ivy1
1Division of Cardiology, Heart Institute, Children's Hospital Colorado, University of Colorado Denver, Anschutz Medical Campus, 13123 E 16th Avenue, Aurora, CO, USA.
Insights
Main pulmonary artery stiffness is increased in children with pulmonary arterial hypertension and impacts right ventricular function. Increased pulse wave velocity predicts clinical outcomes, serving as a potential prognostic marker.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Pulmonary Hypertension Research
Background:
- Pulmonary arterial hypertension (PAH) involves main pulmonary artery (MPA) stiffness and abnormal hemodynamics, increasing right ventricular (RV) afterload.
- The long-term impact of MPA stiffness on RV function in pediatric PAH is not well understood.
Purpose of the Study:
- To comprehensively evaluate MPA stiffness in pediatric PAH.
- To determine the relationship between hemodynamics, MPA stiffness, and RV function.
- To assess the prognostic value of these measures in pediatric PAH.
Main Methods:
- Cardiac magnetic resonance (CMR) was used to assess MPA stiffness and wall shear stress (WSS) in 56 children with PAH and 23 controls.
- Phase contrast CMR evaluated pulse wave velocity (PWV) and relative area change (RAC).
- Prognostic potential was evaluated alongside standard RV volumetric and functional indices.
Main Results:
- Children with PAH showed significantly increased PWV (2.8 m/s vs. 1.4 m/s) and decreased RAC (25% vs. 37%) compared to controls.
- Decreased WSS correlated with a decline in RAC over time (r=0.679).
- Each unit increase in PWV was associated with a 3.2-fold increase in moderate clinical events.
Conclusions:
- Non-invasive CMR assessment revealed increased MPA stiffness in pediatric PAH, correlating with impaired RV performance.
- PWV is a significant predictor of clinical outcomes and a potential prognostic marker in pediatric PAH.
Aims:
Main pulmonary artery (MPA) stiffness and abnormal flow haemodynamics in pulmonary arterial hypertension (PAH) are strongly associated with elevated right ventricular (RV) afterload and associated with disease severity and poor clinical outcomes in adults with PAH. However, the long-term effects of MPA stiffness on RV function in children with PAH remain poorly understood. This study is the first comprehensive evaluation of MPA stiffness in children with PAH, delineating the mechanistic relationship between flow haemodynamics and MPA stiffness as well as the prognostic ability of these measures regarding clinical outcomes.
Methods And Results:
Fifty-six children diagnosed with PAH underwent baseline cardiac magnetic resonance (CMR) acquisition and were compared with 23 control subjects. MPA stiffness and wall shear stress (WSS) were evaluated using phase contrast CMR and were evaluated for prognostic potential along with standard RV volumetric and functional indices. Pulse wave velocity (PWV) was significantly increased (2.8 m/s vs. 1.4 m/s, P < 0.0001) and relative area change (RAC) was decreased (25% vs. 37%, P < 0.0001) in the PAH group, correlating with metrics of RV performance. Decreased WSS was associated with a decrease in RAC over time (r = 0.679, P < 0.001). For each unit increase in PWV, there was approximately a 3.2-fold increase in having a moderate clinical event.
Conclusion:
MPA stiffness assessed by non-invasive CMR was increased in children with PAH and correlated with RV performance, suggesting that MPA stiffness is a major contribution to RV dysfunction. PWV is predictive of moderate clinical outcomes, and may be a useful prognostic marker of disease activity in children with PAH.
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