Extension of BPS-MR-PAH-203 in Pulmonary Arterial Hypertension (PAH) Patients

Eligible patients who participated in BPS-MR-PAH-203 and who elect to continue receiving
study drug in an open-label extension.Each patient will return to the clinic following
enrollment in the study at 3, 6, and 12 months, and annually thereafter for assessment.
Patients will be called by study personnel to assess adverse events and concomitant
medications at Month 9, and at 3 month intervals following the annual visit.

At the End of Study visit, patients discontinuing study drug will be down-titrated off of
BPS-MR at the discretion of the Investigator, at a maximum decrement of one tablet (60µg)
b.i.d. per day and a minimum decrement of one tablet (60µg) b.i.d. per week. Likewise,
patients who withdraw early from the study will be down-titrated off of BPS-MR in the same
manner. Upon completion of down-titration, patients will return to the clinic for a final
Closeout visit.