Abstract
Despite recent advances in Pulmonary Arterial Hypertension (PAH) treatment, this condition is still characterized by an extremely poor prognosis. In this review, we discuss the use of newly-approved drugs for PAH treatment with already known mechanisms of action (macitentan), innovative targets (riociguat and selexipag), and novel therapeutic approaches with initial up-front combination therapy. Secondly, we describe new potential signaling pathways and investigational drugs with promising role in the treatment of PAH.
Keywords: Pulmonary arterial hypertension, macitentan, riociguat, selexipag, initial combination therapy, gene therapy, serotonin, inflammation, immunity, metabolism, pulmonary arterial denervation.
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