title: Existing drugs and agents under investigation for pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension is a progressive and debilitating disorder with an associated high morbidity and mortality rate. Significant advances in our understanding of the epidemiology, pathogenesis, and pathophysiology of pulmonary hypertension have occurred over the past several decades. This has allowed the development of new therapeutic options in this disease. Today, our selection of therapeutic modalities is broader, including calcium channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase inhibitors, and soluble guanylate cyclase stimulators, but the disease remains fatal. This underscores the need for a continued search for novel therapies. Several potential pharmacologic agents for the treatment of pulmonary arterial hypertension are under clinical development and some promising results with these treatments have been reported. These agents include rho-kinase inhibitors, long-acting nonprostanoid prostacyclin receptor agonists, tyrosine protein kinase inhibitors, endothelial nitric oxide synthase couplers, synthetically produced vasoactive intestinal peptide, antagonists of the 5-HT2 receptors, and others. This article will review several of these promising new therapies and will discuss the current evidence regarding their potential benefit in pulmonary arterial hypertension.
authors.affiliation: From the *Department of Medicine, Division of Cardiology, New York Medical College, Westchester Medical Center, Valhalla, NY; and †Department of Medicine, Yale School of Medicine/Norwalk Hospital, Norwalk, CT.
authors.forename: Mala
authors.initials: M
authors.lastname: Sharma
authors.forename: Sowmya
authors.initials: S
authors.lastname: Pinnamaneni
authors.forename: Wilbert S
authors.initials: WS
authors.lastname: Aronow
authors.forename: Bartosz
authors.initials: B
authors.lastname: Jozwik
authors.forename: William H
authors.initials: WH
authors.lastname: Frishman
mesh_terms: D018674:Adrenergic Antagonists / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D065092:Antidiuretic Hormone Receptor Antagonists / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D055808:Drug Discovery / Q000379:methods; D015507:Drugs, Investigational / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000451:Mineralocorticoid Receptor Antagonists / Q000627:therapeutic use; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D044006:Receptors, Epoprostenol / Q000037:antagonists & inhibitors; D012702:Serotonin Antagonists / Q000627:therapeutic use; D033581:Stem Cell Transplantation / Q000379:methods; D066253:Vascular Remodeling / Q000187:drug effects; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D018674:Adrenergic Antagonists; D000893:Anti-Inflammatory Agents; D065092:Antidiuretic Hormone Receptor Antagonists; D000959:Antihypertensive Agents; D015507:Drugs, Investigational; D007166:Immunosuppressive Agents; D000451:Mineralocorticoid Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D047428:Protein Kinase Inhibitors; D044006:Receptors, Epoprostenol; D012702:Serotonin Antagonists; D014665:Vasodilator Agents