A Study to Find Out if Selexipag is Effective and Safe in Patients With Chronic Thromboembolic Pulmonary Hypertension When the Disease is Inoperable or Persistent/Recurrent After Surgery and/or Interventional Treatment
Condition: Chronic Thromboembolic Pulmonary Hypertension
MeSH terms: Hypertension, Pulmonary; Hypertension
Intervention: Selexipag; Placebo
Intervention description: oral tablets containing 200 µg of selexipag. Depending on the iMTD, participants will receive 1 to 8 tablets at each administration; Oral tablets without active compound
Intervention MeSH terms: Selexipag
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Allocation: Randomized
Intervention model: Sequential Assignment
Masking: Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor)
Enrollment: 128.0
Sex: All
Minimum age: 18 Years
Maximum age: 85 Years
Healthy volunteers: No
Keywords: CTEPH; selexipag

Summary:

      Selexipag is available in many countries for the treatment of pulmonary arterial hypertension
      (PAH). Due to the similarities between PAH and chronic thromboembolic pulmonary hypertension
      (CTEPH) and the observed efficacy of other PAH medicines in CTEPH, it is believed that
      selexipag could benefit to patients with CTEPH. This study aims to assess the efficacy and
      safety of selexipag in participants with inoperable or persistent/recurrent CTEPH.
    

Eligibility criteria:

        Main Inclusion Criteria:

          -  Signed and dated informed consent form

          -  Male and female participants from greater than or equal to (>) 18 (or the legal age of
             consent in the jurisdiction in which the study is taking place) and less then or equal
             to (<=85) years old at Screening (Visit 1)

          -  With established diagnosis of inoperable CTEPH (i.e., technically non-operable) or
             persistent/recurrent CTEPH after pulmonary endarterectomy (PEA) and/or balloon
             pulmonary angioplasty (BPA), as confirmed by the corresponding adjudication committee

          -  With pulmonary hypertension (PH) in WHO FC I-IV.

          -  Participant able to perform the 6-minute walk test (6MWT) with a minimum distance of
             100 m and a maximum distance of 450 m at screening visit.

          -  Women of childbearing potential must have a negative pregnancy test at screening and
             randomization and must agree to undertake monthly urine pregnancy tests, and to use a
             reliable method of birth control from screening visit up to at least 30 days after
             study treatment discontinuation. If a hormonal contraceptive is chosen it must be
             taken for at least 1 month prior to randomization.

        Main Exclusion Criteria:

          -  Planned or current treatment with another investigational treatment up to 3 months
             prior to randomization.

          -  Any known factor or disease that might interfere with treatment compliance, study
             conduct, or interpretation of the results, such as drug or alcohol dependence or
             psychiatric disease.

          -  Known concomitant life-threatening disease with a life expectancy < 12 months.

          -  Planned balloon pulmonary angioplasty within 26 weeks after randomization.

          -  Change in dose or initiation of new PH-specific therapy within 90 days prior to the
             baseline RHC (and LHC, if needed) qualifying for enrollment for the hemodynamic cohort
             and within 90 days prior to randomization (Visit 2) for the non-hemodynamic cohort

          -  Treatment with prostacyclin (epoprostenol), prostacyclin analogs (i.e., treprostinil,
             iloprost, beraprost) or prostacyclin receptor agonists (i.e., selexipag) within 90
             days prior to randomization (visit 2) except those given at vasodilator testing during
             RHC

          -  Change in dose or initiation of new diuretics and/or calcium channel blockers within 1
             week prior to baseline RHC (and LHC, if needed)

          -  Any co-morbid condition that may influence the ability to perform a reliable and
             reproducible 6MWT, including use of walking aids (cane, walker, etc).

          -  Any other criteria as per selexipag Summary of Product Characteristics (SmPC).

          -  Exclusion criteria related to comorbidities: severe coronary heart disease or unstable
             angina as assessed by the investigator; mocardial infarction within the last 6 months
             prior to or during Screening; decompensated cardiac failure if not under close
             supervision; severe arrhythmias as assessed by the investigator; cerebrovascular
             events (example transient ischemic attack, stroke) within the last 3 months prior to
             or during screening; congenital or acquired valvular defects with clinically relevant
             myocardial function disorders not related to pulmonary hypertension. (PH); known or
             suspicion of pulmonary veno-occlusive disease