Key result
This review summarizes key trials leading to the FDA approval of macitentan, riociguat, and oral treprostinil diolamine for the treatment of pulmonary arterial hypertension.
Why the study?
New medications including macitentan, riociguat, and treprostinil diolamine have been recently approved for group 1 pulmonary arterial hypertension, necessitating an updated review of their clinical use and combination therapy strategies.
This review summarizes the key trials and clinical considerations for newly approved medications (macitentan, riociguat, and oral treprostinil) for pulmonary arterial hypertension.
Pulmonary arterial hypertension is a chronic, progressive disease of the pulmonary vasculature resulting in poor outcomes if left untreated. The management of group 1 pulmonary arterial hypertension has included the use of prostanoids, phosphodiesterase-5 inhibitors, and endothelin receptor antagonists targeting the prostacyclin, endothelin-1, and nitric oxide pathways. Three new medications have been approved by the US Food and Drug Administration over the past couple of years. Macitentan is the newest endothelin receptor antagonist, riociguat is a soluble guanylate cyclase stimulator, and treprostinil diolamine is the first oral prostanoid. This review will focus on the key trials leading to their approval, special considerations for each medication, and their potential place in therapy. The use of combination therapy as initial therapy in pulmonary arterial hypertension will also be discussed.
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Enderby et al. (2015) conducted a review in Pulmonary arterial hypertension. Macitentan, riociguat, and treprostinil diolamine was evaluated. This review summarizes key trials leading to the FDA approval of macitentan, riociguat, and oral treprostinil diolamine for the treatment of pulmonary arterial hypertension.
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