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Related Experiment Video

Updated: Jul 1, 2026

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Published on: June 27, 2025

Right ventricular function in scleroderma-related pulmonary hypertension.

A Vonk Noordegraaf¹, R Naeije

¹Department of Pulmonary Diseases, Institute for Cardiovascular Research, VU University Medical Center, Amsterdam, The Netherlands. A.Vonk@VUmc.nl

Rheumatology (Oxford, England)

|September 17, 2008

Summary

Pulmonary arterial hypertension in systemic sclerosis (SSc-PAH) shows poorer outcomes, with distinct right ventricular (RV) dysfunction compared to idiopathic PAH. Further research is needed to understand the RV

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Published on: June 15, 2020

Area of Science:

Cardiology
Rheumatology
Pulmonary Medicine

Background:

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) presents a poorer prognosis than other forms of pulmonary hypertension.
Emerging evidence suggests unique alterations in right ventricular (RV) function and filling in SSc-PAH compared to idiopathic PAH.

Purpose of the Study:

To investigate the potential reasons behind the increased vulnerability of the right ventricle in SSc-PAH.
To explore the roles of intrinsic myocardial disease, coronary vasculopathy, and altered pulmonary arterial compliance in RV dysfunction in SSc-PAH.

Main Methods:

Review of recent echocardiographic and hemodynamic studies.
Analysis of characteristic impedance measurements to assess pulmonary arterial compliance.
Consideration of pathological and imaging data.

Main Results:

SSc-PAH patients exhibit altered RV pump function and filling characteristics.
Potential contributing factors include myocardial fibrosis, coronary vasculopathy, and reduced pulmonary arterial compliance.
RV-arterial decoupling may be exacerbated by decreased pulmonary arterial compliance.

Conclusions:

The right ventricle appears uniquely vulnerable in SSc-PAH.
Intrinsic myocardial disease and vascular factors likely contribute to RV dysfunction.
Further pathological, imaging, and hemodynamic studies are essential to fully elucidate RV vulnerability in SSc-PAH.