Detection Of Latent Pulmonary Hypertension In Genetically Susceptible Individuals.

Recruiting

Conditions: 10019280
10083624
10037454
early disease
pulmonary hypertension

Registration Number: NL-OMON53083

Lead Sponsor: Vrije Universiteit Medisch Centrum

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 140

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 <80 years
3. Able to understand and willing to sign the Informed Consent Form

Exclusion Criteria

- Pregnant subjects
- Claustrophobia
- Inability to provide informed consent
- In case of PAH patients: TLC < 70%pred or radiographic evidence of
interstitial lung disease
- In case of BMPR2 mutation carriers and family control subjects, one or more
of the following: abnormal spirometry, TLC < 70%, echocardiographic evidence of
pulmonary hypertension

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>-Platelet markers (RNA profiles)<br /><br>-Metabolites and cytokines markers<br /><br>-Exosome markers<br /><br>-Change in mean pulmonary artery pressure (mPAP)<br /><br>-Change in cardiac output (CO)<br /><br>-Change in right atrial pressure (RAP)<br /><br>-Change in right ventricular ejection fraction (RVEF) (%)<br /><br>-Change in right ventricular end diastolic volume (RVEDV) (ml)<br /><br>-Change in right ventricular end systolic volume (RVESV) (ml)<br /><br>-Change in CPET markers: maximal work (VE), peak oxygen consumption (VO2),<br /><br>maximal oxygen pulse (O2-pulse), VO2 max, anaerobic threshold (AT), ventilation<br /><br>for a given volume of carbon dioxide production (VE/VCO2-slope) and the<br /><br>end-tidal CO2 tension (PET-CO2).<br /><br>-Change in 6 minute walking distance<br /><br>- Changes in pulmonary vasculature<br /><br>Density and abscence of pulmonary vasculature<br /><br>Abnormal or abberant morphology of pulmonary vasculature</p><br>

Secondary Outcome Measures