Conditions: Pulmonary Arterial Hypertension
Keywords: bosentan PAH related to Eisenmenger physiology Tracleer
Allocation: NON_RANDOMIZED | Intervention model: SINGLE_GROUP
Enrollment: 37
Healthy volunteers: no

Brief summary:
This 6-month open label study will evaluate the long term safety of bosentan (via oxygen saturation) and efficacy (exercise capacity) in patients who have completed the BREATHE-5 study (PAH related to Eisenmenger physiology). Treatment duration is 6 months.

Detailed description:
This is a multicenter, open-label, single -arm study with bosentan, initial dose of 62.5 mg b.i.d., with a target dose of 125 mg b.i.d. All patients will be assessed for eligibility during the baseline visit (same as Week 16, end of study BREATHE-5 visit), and will have the option to enter into the open-label extension study at a starting dose of 62.5 mg b.i.d., for safety reasons. Visit 2 is required as a safety visit to assess oxygen saturation (SpO2) after 1 week of bosentan treatment. Patients will return for Visit 4 after 4 weeks of treatment, and the dose will be up-titrated to 125 mg b.i.d. or maintained at 62.5 mg b.i.d. at the judgement of the investigator. Data will be collected for a total of 24 weeks or until the sponsor decides to stop the study. If the results from Study AC-052-403 or AC-052-405 demonstrate significant safety issues, this extension study may be terminated at the sponsor's request.

Arms:
arm 1: The starting dose for all patients will be 62.5 mg b.i.d. At the Week 4 visit, patients who were started on 62.5 mg b.i.d. will be uptitrated to 125 mg b.i.d. if the 62.5 mg b.i.d. dose was well-tolerated.

Interventions: intervention 1: Tracleer®
Intervention descriptions: intervention 1: Patients will receive up to 125 mg b.i.d. of Tracleer.

Protocol document: