early disease pulmonary hypertension
Conditions
Interventions
None listed
Sponsors
Vrije Universiteit Medisch Centrum
Eligibility
Age
16 Years to 64 Years
Inclusion criteria
Inclusion criteria: 1. Diagnosis of idiopathic PAH, according to ESC/ERS guidelines (ref: Galie ERJ 2015) Or Unaffected BMPR2 or other mutation carrier or other mutations associated with PAH Or Healthy relative of heritable PAH patient not carrying the disease causing mutation 2. Age >18 and
Exclusion criteria
Exclusion criteria: - Pregnant subjects - Claustrophobia - Inability to provide informed consent - In case of PAH patients: TLC
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| -Platelet markers (RNA profiles) -Metabolites and cytokines markers -Exosome markers -Change in mean pulmonary artery pressure (mPAP) -Change in cardiac output (CO) -Change in right atrial pressure (RAP) -Change in right ventricular ejection fraction (RVEF) (%) -Change in right ventricular end diastolic volume (RVEDV) (ml) -Change in right ventricular end systolic volume (RVESV) (ml) -Change in CPET markers: maximal work (VE), peak oxygen consumption (VO2), maximal oxygen pulse (O2-pulse), VO2 max, anaerobic threshold (AT), ventilation for a given volume of carbon dioxide production (VE/VCO2-slope) and the end-tidal CO2 tension (PET-CO2). -Change in 6 minute walking distance - Changes in pulmonary vasculature Density and abscence of pulmonary vasculature Abnormal or abberant morphology of pulmonary vasculature | — |
Secondary
| Measure | Time frame |
|---|---|
| non | — |
Countries
Netherlands
Outcome results
None listed