Therapeutic advances in the field of pulmonary arterial hypertension (PAH) have been considerable over the past 2 decades. We now have numerous US Food and Drug Administration-approved therapies that fall into three classes: the prostanoids, the endothelin receptor antagonists, and the phosphodiesterase type 5 inhibitors. Therapies in several novel therapeutic classes are being investigated.
The prostanoids are among the most effective therapies, particularly for patients with more advanced disease. They are, however, hindered by cumbersome delivery systems requiring continuous IV infusion (epoprosentol and treprostinil), continuous subcutaneous infusion (treprostinil), or frequent interment inhalation (iloprost and treprostinil). The “holy grail” of prostanoid therapy would be an effective, well-tolerated, oral form. Beraprost is an oral prostanoid that is currently approved only in Japan. Randomized controlled trial data with beraprost are contradictory, with one trial demonstrating an improvement in 6-min walk distance (6MWD) at 12 weeks and another establishing that beraprost failed to prevent disease progression at 1 year.1,2