title: Update on pulmonary arterial hypertension pharmacotherapy.
abstract: Pulmonary artery hypertension (PAH) refers to several subgroups of disease in which the mean pulmonary artery pressure (mPAP) is elevated to more than 25 mm Hg, pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg, and an elevated pulmonary vascular resistance (PVR) > 3 Wood units as confirmed by right heart catheterization. The prevalence and geographic distribution of PAH vary depending on the type and etiology of the disease. Despite enormous efforts in the research and development of therapeutic agents in the last twenty years, the disease remains relatively incurable and the overall prognosis remains guarded. Median survival for an untreated patient is 2.8 years. In the last three decades, there have been dramatic advances in understanding the molecular mechanisms and signaling pathways involved in the disease, resulting in emerging new treatment strategies. In the following pages, we will review currently approved treatments for PAH, as well as a new generation of investigational drugs.
authors.affiliation: a Department of Medicine, Division of Cardiology , Westchester Medical Center/New York Medical College , Valhalla , NY , USA.
authors.forename: Arash
authors.initials: A
authors.lastname: Velayati
authors.affiliation: a Department of Medicine, Division of Cardiology , Westchester Medical Center/New York Medical College , Valhalla , NY , USA.
authors.forename: Marcos G
authors.initials: MG
authors.lastname: Valerio
authors.affiliation: a Department of Medicine, Division of Cardiology , Westchester Medical Center/New York Medical College , Valhalla , NY , USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Shen
authors.affiliation: a Department of Medicine, Division of Cardiology , Westchester Medical Center/New York Medical College , Valhalla , NY , USA.
authors.forename: Sohaib
authors.initials: S
authors.lastname: Tariq
authors.affiliation: a Department of Medicine, Division of Cardiology , Westchester Medical Center/New York Medical College , Valhalla , NY , USA.
authors.forename: Gregg M
authors.initials: GM
authors.lastname: Lanier
authors.affiliation: a Department of Medicine, Division of Cardiology , Westchester Medical Center/New York Medical College , Valhalla , NY , USA.
authors.forename: Wilbert S
authors.initials: WS
authors.lastname: Aronow
mesh_terms: D002121:Calcium Channel Blockers / Q000627:therapeutic use; D003999:Dichloroacetic Acid / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011651:Pulmonary Artery; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D044006:Receptors, Epoprostenol / Q000037:antagonists & inhibitors*; D012042:Registries; D000071756:Soluble Guanylyl Cyclase / Q000378:metabolism; D054460:rho-Associated Kinases / Q000037:antagonists & inhibitors
chemical_list: D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D047428:Protein Kinase Inhibitors; D011720:Pyrazoles; D011743:Pyrimidines; D044006:Receptors, Epoprostenol; D003999:Dichloroacetic Acid; D011464:Epoprostenol; D054460:rho-Associated Kinases; D000071756:Soluble Guanylyl Cyclase; C542595:riociguat
keywords: CTEPH; Imatinib; PAH; PDE-5i; PH; Pulmonary hypertension; ambrisentan; amlodipine sildenafil; beraprost; bosentan; cyclic AMP; dichloroacetate; diltiazem; epoprostenol; guanylate cyclase; iloprost; macitentan; nifedipine; nitric oxide; prostacyclin; ranolazine; selexipag; tadalafil; trimetazidine; vardenafil riociguat