Valentina Mercurio*, Anna Bianco, Giacomo Campi, Alessandra Cuomo, Nermin Diab, Angela Mancini, Paolo Parrella, Mario Petretta, Paul M. Hassoun and Domenico Bonaduce Pages 2844 - 2864 ( 21 )
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.
Pulmonary arterial hypertension, macitentan, riociguat, selexipag, initial combination therapy, gene therapy, serotonin, inflammation, immunity, metabolism, pulmonary arterial denervation.
Federico II University, Department of Translational Medical Sciences, Naples, Federico II University, Department of Translational Medical Sciences, Naples, Federico II University, Department of Translational Medical Sciences, Naples, Federico II University, Department of Translational Medical Sciences, Naples, University of Ottawa, Department of Medicine, Ottawa, ON, Federico II University, Department of Translational Medical Sciences, Naples, Federico II University, Department of Translational Medical Sciences, Naples, Federico II University, Department of Translational Medical Sciences, Naples, Johns Hopkins University, Division of Pulmonary and Critical Care Medicine, Baltimore, MD, Federico II University, Department of Translational Medical Sciences, Naples