Chronic thromboembolic pulmonary hypertension
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: Patients must be >=18 years old, able to provide informed consent, and receive care for CTEPH at Kyushu University Hospital between study approval and March 31, 2028. Necessary condition Reduced exercise capacity (peak VO2 <15 mL/min/kg) with residual pulmonary hypertension (mPAP >20 mmHg), and prior PEA or BPA with no indication for further intervention, requiring pulmonary vasodilator therapy (riociguat or selexipag). Sufficient condition (at least 1 required) ?Inadequate response to riociguat after >=90 days (peak VO2 <=15 mL/min/kg and mPAP >20 mmHg) ?Documented reason not to use riociguat (e.g., hypotension, lung disease, renal dysfunction, adverse effects, or prior inefficacy) Note Selexipag initiation is based on clinical indication and is not influenced by the study.
Exclusion criteria
Exclusion criteria: (1) Unable to undergo MRI, cannot tolerate exercise stress, or otherwise unable to complete the study procedures (2) Pregnant patients (3) Dementia (4) Any patient deemed inappropriate for study participation by the investigator
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in SV, RVEDV/LVEDV, and RVESV/LVESV on exCMR before and after selexipag. | — |
Secondary
| Measure | Time frame |
|---|---|
| Change in WHO-FC, 6MWD, CPX parameters, emPHasis-10, and NT-proBNP | — |
Countries
Japan
Contacts
Kyushu University Graduate School of Medicines Department of Cardiovascular Medicine