The FDA has removed the Risk Evaluation and Mitigation Strategy (REMS) requirement for the endothelin receptor antagonists ambrisentan (Letairis, and generics), bosentan (Tracleer, and generics), macitentan (Opsumit, and generics; Opsynvi), and aprocitentan (Tryvio), and the endothelin receptor antagonist and angiotensin II receptor antagonist sparsentan (Filspari) that was initially implemented because of a possible risk of embryofetal toxicity.

The FDA has approved sotatercept-csrk (Winrevair – Merck), a first-in-class activin signaling inhibitor, to increase exercise capacity, improve WHO functional class, and reduce the risk of clinical worsening events in adults with WHO Group 1 pulmonary arterial hypertension (PAH).

The FDA has approved selexipag (Uptravi – Actelion), an oral selective prostacyclin IP receptor agonist, for treatment of pulmonary arterial hypertension (PAH). Treprostinil (Orenitram), an oral prostacyclin analog, was approved earlier for this indication.

The FDA has approved the use of ambrisentan (Letairis) and tadalafil (Adcirca) together for treatment of pulmonary arterial hypertension (PAH). It is the first 2-drug regimen to be approved for this indication.

The FDA has approved the sGC stimulator riociguat (rye" oh sig' ue at; Adempas – Bayer) for oral treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) following surgery or when surgery is not an option. It is the first drug to be approved for treatment of CTEPH.

The FDA has approved macitentan (ma" si ten' tan; Opsumit – Actelion), for oral treatment of pulmonary arterial hypertension (PAH). Macitentan is the second nonselective endothelin receptor antagonist approved for PAH. It is a derivative of bosentan (Tracleer), which is also manufactured by Actelion, and is scheduled to become available generically in 2015. Riociguat (Adempas), another new drug for this indication, will be reviewed in a future issue. Volibris

The FDA has approved use of tadalafil (Adcirca - Lilly/United Therapeutics) for treatment of pulmonary arterial hypertension (PAH).

Ambrisentan (Letairis - Gilead), a selective endothelin type A (ET_A) receptor antagonist, has been approved by the FDA for treatment of symptomatic patients (WHO class II or III) with pulmonary arterial hypertension (PAH).