FUTURE 3 Study Extension

Phase 3

Completed

Brief Summary: The objectives of the FUTURE 3 Study Extension are to evaluate the long-term safety, tolerability and efficacy of the pediatric formulation of bosentan two versus three times a day in children with Pulmonary Arterial Hypertension (PAH).

Recruitment & Eligibility

Inclusion Criteria

Patients who completed the FUTURE 3 core study (AC-052-373) or prematurely discontinued due to PAH-progression, if bosentan was not permanently discontinued
Patients who tolerated bosentan pediatric formulation and for whom bosentan is considered beneficial at the end of the FUTURE 3 core study (AC-052-373)
Signed informed consent by the parents or the legal representatives prior to any study-mandated procedure.

Exclusion Criteria

Known intolerance or hypersensitivity to bosentan or any of the excipients of the dispersible bosentan tablet
Any clinically significant laboratory abnormality that precludes continuation of bosentan therapy
Pregnancy
AST and/or ALT values > 3 times the upper limit of normal range (ULN)
Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C
Premature and permanent study drug discontinuation during the FUTURE 3 core study (AC-052-373)
Any major violation of the FUTURE 3 core study (AC-052-373) protocol.

Arm && Interventions

Group	Intervention	Description
bosentan 2mg/kg b.i.d.	Bosentan	Patients who received 2 mg/kg bosentan twcie daily (b.i.d.) during the FUTURE 3 core study and continued with the same dose regimen during the extension study
bosentan 2mg/kg t.i.d.	Bosentan	Patients who received 2 mg/kg bosentan 3 times a day (t.i.d.) during the FUTURE 3 core study and continued with the same dose regimen during the extension study

Primary Outcome Measures

Name	Time	Method
Treatment Emergent Adverse Events (AEs) up to 7 Days After Permanent Study Drug Discontinuation	Up to 62 weeks in average	This is the total number of subjects with at least one adverse event (serious or not serious) whether or not causally related to the study drug and presented cumulatively in the FUTURE 3 and FUTURE 3 Extension study. NOTE: FUTURE 3 extension study was exploratory and no primary efficacy and safety endpoints were defined in the protocol. So, this safety outcome measure was selected and reported as primary endpoint here.

Secondary Outcome Measures

Name	Time	Method

Locations (36): The Children's Hospital - Site 9102
🇺🇸
Aurora, Colorado, United States
Children's National Medical Center - Site 9104
🇺🇸
Washington, District of Columbia, United States
Columbia University Medical Center Children's Hospital of New York Presbyterian - Site 9101
🇺🇸
New York, New York, United States
Royal Children's Hospital Melbourne, Cardiology - Site 5001
🇦🇺
Parkville, Australia
The Republican Scientific-Practical Center "Cardiology" - Site 3001
🇧🇾
Minsk, Belarus
Cardiovascular Institute and Fuwai Hospital
🇨🇳
Beijing, China
Shanghai Children's Medical Center - Site 5102
🇨🇳
Shanghai, China
Fakultní nemocnice v Motole, dětské kardiocentrum - Site 3301
🇨🇿
Prague, Czechia
Hopital Necker-Enfants Malades, Service de Cardiologie Pédiatrique - Site 2201
🇫🇷
Paris, France
CHU de Toulouse - Hôpital des Enfants, Service de Cardiologie Pédiatrique - Site 2202
🇫🇷
Toulouse, France
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The Children's Hospital - Site 9102
🇺🇸Aurora, Colorado, United States