A 12-month research project for children who have gone through the FUTURE 5 research project and who want to continue treatment to see how well bosentan is tolerated when administered over a long period of time.

Phase 1

Conditions: Pulmonary arterial hypertension (PAH) in children
MedDRA version: 14.1Level: LLTClassification code 10064908Term: Associated with (APAH)System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders
MedDRA version: 14.1Level: LLTClassification code 10064909Term: Idiopathic (IPAH)System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders
MedDRA version: 14.1Level: LLTClassification code 10064910Term: Familial (FPAH)System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders
Therapeutic area: Diseases [C] - Cardiovascular Diseases [C14]

Registration Number: EUCTR2011-004612-31-NL

Lead Sponsor: Actelion pharmaceuticals Ltd

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: A

Sex: All

Target Recruitment: 105

Inclusion Criteria

1. Signed informed consent by the parents / legal representatives prior to any study-mandated procedure.

2. Patients who:
- were treated for 12 weeks in FUTURE 5
Or
- discontinued FUTURE 5 due to PAH-progression.

And who performed the Visit 5 assessments of FUTURE 5 according to the FUTURE 5 protocol.

3. Patients who did not prematurely discontinue the study drug due to AEs in FUTURE 5.

4. Females of childbearing potential must have a negative pre treatment serum pregnancy test and must use reliable methods of contraception during the whole treatment period and for 1 month after study drug discontinuation.
Are the trial subjects under 18? yes
Number of subjects for this age range: 105
F.1.2 Adults (18-64 years) no
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

1. Known intolerance or hypersensitivity to bosentan or any of the excipients of the bosentan dispersible tablet.

2. Pregnancy or breastfeeding.

3. Any AST and/or ALT values > 3 times the upper limit of normal range (ULN) until Visit 5 of FUTURE 5 (inclusive).

4. Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C until Visit 5 of FUTURE 5 (inclusive).

5. Any condition that prevents compliance with FUTURE 6 protocol or adherence to therapy.

Study & Design

Study Type: Interventional clinical trial of medicinal product

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Main Objective: To evaluate the long-term safety and tolerability of the pediatric formulation of bosentan at a dose of 2 mg/kg b.i.d. in children with pulmonary arterial hypertension (PAH).;Secondary Objective: Not Applicable;Primary end point(s): Not applicable;Timepoint(s) of evaluation of this end point: Not applicable

Secondary Outcome Measures