New Therapeutic Paradigms and Guidelines in the Management of Pulmonary Arterial Hypertension.

The FREEDOM-C1 and FREEDOM-C2 studies showed that oral treprostinil (up to 16 mg twice daily) used with background PAH therapy of other classes did not reach statistical significance in the primary endpoint, 6MWD. 53 , 54 In the FREEDOM-M trial, treprostinil, when used in treatment-naïve patients as monotherapy at a dose of up to 12 mg twice daily, significantly improved 6MWD; however, no significant improvement in clinical worsening or symptoms was demonstrated. 55 Adverse effects of treprostinil are headache, nausea, diarrhea, flushing, jaw pain, hypokalemia, and abdominal pain. 53 Selexipag is a novel agent approved by the FDA in December 2015 to delay disease progression and reduce the risk of hospitalization for PAH.

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