SERAPHIN-OL: Study with an ERA in Pulmonary arterial Hypertension to Improve cliNical outcome (Open Label)Long-term single-arm open-label extension study of the SERAPHIN study, to assess the safety and tolerability of macitentan patients with symptomatic pulmonary arterial hypertension
This is a multicenter, double-blind, randomized, placebo-controlled, parallel group, event-driven, Phase iii study to assess the effects of macitentan on morbidity and mortality in patients with symptomatic pulmonary arterial hypertension.
The target population of the study will be patients with PaH in the absence of a demonstrable cause (idiopathic or familial), or associated with collagen vascular disease, simple congenital systemic to pulmonary shunts surgically corrected, or HiV infection. The study will enroll males or females aged 12 years or older with symptomatic PaH, who may benefit from receiving macitentan as monotherapy or in combination with existing PaH therapy, but this site will enroll patients [GreaterThanorequalTo] 18 years of age to [LessThanorequalTo] 85 years of age. Patients randomized in this study must have a diagnosis of PaH proven by right heart catheterization performed within 12 months prior to randomization, and must be within WHo functional class ii to iV.
Patients will be randomized 1:1:1 to one of three groups: placebo, macitentan 3 mg or macitentan 10 mg. our site will enroll up to to 12 patients. The primary study end-point will be time to first morbidity (clinical worsening; see biostatistics section) or mortality event.
1. Signed informed consent prior to initiation of any study-mandated procedure.
2. Patients with pulmonary arterial hypertension and having completed the event-driven study, AC-055-302/SERAPIN, or
3. Patients who have experienced a morbidity event and for whom a written approval to roll over into this study has been obtained from the Sponsor.