Amsterdam/Aarhus Chronic Thromboembolic Pulmonary Hypertension / Chronic Thromboembolic Disease (CTEPH/CTED) Cohort (2A3C) Study<br>

Recruiting

Conditions: Chronic Thromboembolic Pulmonary Hypertension
10037454

Registration Number: NL-OMON47632

Lead Sponsor: Vrije Universiteit Medisch Centrum

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 200

Inclusion Criteria

- Diagnosis of CTEPH or CTED (according to ESC/ERS guidelines (11))
- Scheduled for evaluation to determine possibility of PEA or BPA
- Age > 18

Exclusion Criteria

- Pacemaker or other metal objects in the body
- Severe claustrophobia
- Severe renal insufficiency (eGFR < 30 mL/min)
- Allergy to gadolinium contrast
- Inability to provide informed consent

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Main study parameters/endpoints: This is an observational study evaluating<br /><br>determinants of a persistent response to BPA or PEA. Main study<br /><br>parameter/endpoint is change in extracellular volume fraction after unloading<br /><br>of the RV after PEA or BPA. This is measured calculated from pre and post<br /><br>contrast T1 relaxation times measured by MR imaging.<br /><br></p><br>

Secondary Outcome Measures

Name	Time	Method
<p>- Change in RV function and volumes after PEA or BPA. This is measured by MR<br /><br>imaging (RV ejection fraction, end diastolic volume, end systolic volume) and<br /><br>right heart catheterization (end systolic elastance, RV-Arterial coupling)<br /><br>- Change in lung perfusion defects before and after PEA measured by MR perfusion<br /><br>- Change in serum levels of biomarkers of fibrosis and extracellular matrix<br /><br>- Change in platelet transcriptomes<br /><br>- Change in exercise capacity (peak oxygen uptake during cardiopulmonary<br /><br>exercise testing, or CPET, and distance walked in 6 minutes) and gas exchange<br /><br>during CPET (ventilatory efficiency, pulse oximetry)<br /><br>- Change in quality of life<br /><br>- Change in diffusion capacity</p><br>