Clinical Study to Assess the Safety, Tolerability and Efficacy of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension

Study Overview
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Brief Summary
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Long-term study to evaluate if macitentan is safe, tolerable and efficient enough to be used for treatment of inoperable chronic thromboembolic pulmonary hypertension (CTEPH)

Official Title
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MERIT-2 : Long Term, Multicenter, Single-arm, Open-label Extension Study of the MERIT-1 Study, to Assess the Safety, Tolerabilty and Efficacy of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Conditions
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Chronic Thromboembolic Pulmonary Hypertension

Intervention / Treatment
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* Drug: Macitentan


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Written informed consent Subject with CTEPH having completed the double-blind (DB) AC-055E201/ MERIT-1 study as scheduled (i.e., who remained in the DB study up to Week 24). Females of childbearing potential must have a negative pre-treatment serum pregnancy test, be advised on appropriate methods of contraception, and agree to use 2 reliable methods of contraception. Exclusion Criteria: Permanent discontinuation of DB study treatment due to an hepatic adverse event or liver aminotransferase abnormalities. Any known factor (e.g., drug or substance abuse) or disease (e.g., unstable psychiatric illness) that, in the opinion of the investigator, may interfere with treatment compliance or interpretation of the results, or that may influence the ability to comply with any of the study requirements.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Macitentan<br>Macitentan 10mg, oral tablet, once daily | Drug: Macitentan<br>* Macitentan 10mg, oral tablet, once daily<br>* Other names: ACT-064992;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of Participants With Treatment-emergent Adverse Events (TEAEs) | An adverse event (AE) is any untoward medical occurrence in a participant participating in a clinical study that does not necessarily have a causal relationship with the pharmaceutical/ biological agent under study. TEAEs are those events that started after administration of the first dose and up to safety follow-up visit/end of study, that is, 30 days after the last dose of study medication. | Up to 30 days after study treatment discontinuation (treatment exposure ranged from 1 to 82 months) | 
| Number of Participants With AEs Leading to Study Drug Discontinuation | Number of participants with AEs leading to study drug discontinuation was reported. | Up to 30 days after study treatment discontinuation (treatment exposure ranged from 1 to 82 months) | 
| Number of Participants With Treatment-emergent Serious Adverse Events (SAEs) | A serious adverse event (SAE) is any untoward medical occurrence that at any dose resulting in any of following outcomes: results in death, is life-threatening, requires inpatient hospitalization or prolongation of existing hospitalization, results in persistent or significant disability/incapacity, is a congenital anomaly/birth defect, is a suspected transmission of any infectious agent via a medicinal product. Treatment-emergent SAEs were those events that started after administration of the first dose and up to safety follow-up visit/end of study, that is, 30 days after the last dose of study medication. | Up to 30 days after study treatment discontinuation (treatment exposure ranged from 1 to 82 months) | 
| Number of Participants With Hemoglobin Abnormalities | Number of participants with hemoglobin abnormalities were reported. It included hemoglobin less than (<) 80 grams per liter (g/L), hemoglobin <100 g/L, hemoglobin greater than or equal to (>=) 80 g/L and <100 g/L, hemoglobin <100g/L and a decrease of >20 g/L from baseline, decrease of >20 g/L in hemoglobin from baseline, decrease of >20 g/L and <=50 g/L in hemoglobin from baseline, and decrease of >50 g/L in hemoglobin from baseline. | Up to 30 days after study treatment discontinuation (treatment exposure ranged from 1 to 82 months) | 
| Number of Participants With Liver Tests Abnormalities | Number of participants with liver tests abnormalities were reported. It included alanine aminotransferase (ALT) or aspartate aminotransferase (AST): >=3 x Upper limit of the normal range (ULN), >=3 and <5 x ULN, >=5 ULN, and >=5 and <8 x ULN, >= 8 x ULN, and total bilirubin >=2 x ULN. | Up to 30 days after study treatment discontinuation (treatment exposure ranged from 1 to 82 months) | 
| Change From Baseline in Blood Pressure at Month 6 | Change from baseline in blood pressure at Month 6 (both systolic blood pressure [SBP] and diastolic blood pressure [DBP]) was reported. | Baseline and Month 6 | 
| Change From Baseline in Pulse Rate at Month 6 | Change from baseline in pulse rate at Month 6 was reported. | Baseline and Month 6 | 
| Change From Baseline in Body Weight at Month 6 | Change from baseline in body weight at Month 6 was reported. | Baseline and Month 6 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Chronic thromboembolic pulmonary hypertension (CTEPH)