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Updated: Jan 20, 2026

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
Published on: June 27, 2025
Diaphragm function does not independently predict exercise intolerance in patients with precapillary pulmonary
Jens Spiesshoefer1, Simon Herkenrath2, Michael Mohr3
1Respiratory Physiology Laboratory, Department of Neurology with Institute for Translational Neurology, University Hospital Muenster, Muenster, Germany jens.spiesshoefer@ukmuenster.de.
Patients with pulmonary hypertension (PH) show diaphragm dysfunction, but it does not independently cause exercise intolerance. Right heart failure, indicated by RVEF and NT-proBNP, is the primary driver of exercise limitation in PH.
Area of Science:
- Cardiology
- Pulmonology
- Exercise Physiology
Background:
- Exercise intolerance is common in precapillary pulmonary hypertension (PH).
- Diaphragm dysfunction is a suspected contributor to exercise limitation in PH.
- Multimodal evaluation of these factors in PH is limited.
Purpose of the Study:
- To investigate diaphragm function in patients with PH.
- To identify determinants of exercise intolerance in PH.
- To assess the independent contribution of diaphragm dysfunction to exercise limitation.
Main Methods:
- 43 PH patients and 43 controls underwent ultrasound for diaphragm function, spirometry, blood gas analysis, echocardiography for pulmonary artery pressure (PAP), NT-proBNP assay, and cardiac MRI for RVEF.
- Exercise capacity was measured by 6-minute walk distance (6MWD).
Main Results:
- PH patients exhibited significantly lower diaphragm excursion velocity and thickening ratio compared to controls.
- Worse exercise tolerance in PH was associated with poorer diaphragm function, higher NT-proBNP, lower CO2, and lower RVEF.
- Multivariate analysis identified RVEF and NT-proBNP as independent predictors of exercise limitation, not diaphragm function.
Conclusions:
- Patients with PH demonstrate diaphragm dysfunction, which worsens with increasing exercise intolerance.
- Diaphragm dysfunction in PH does not independently predict exercise limitation.
- Right heart dysfunction, reflected by RVEF and NT-proBNP, is the primary factor explaining exercise intolerance in PH.
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