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 Hypertension, endothelin receptor, soluble guanylate cyclase, prostanoids, initial combination therapy, gene therapy, serotonin, inflammation, immunity, metabolism, pulmonary arterial denervation.
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