title: Pathways in pulmonary arterial hypertension: the future is here.
abstract: It is well established that the endothelin, nitric oxide and prostacyclin pathways play an important role in the development of pulmonary arterial hypertension (PAH). Indeed, the therapeutic options currently available for the management of PAH all act on one of these mechanistic pathways. However, this is an exciting time for both clinicians and scientists, as increased understanding of the mechanisms involved in the pathogenesis and progression of PAH has resulted in the development of a number of novel therapeutic options. This article highlights how the introduction of new compounds such as macitentan, riociguat and selexipag, which act on the endothelin, nitric oxide and prostacyclin pathways, respectively, have the potential to further improve the prognosis for patients with PAH.
authors.affiliation: Université Paris-Sud, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Nicholasw
authors.initials: N
authors.lastname: Morrell
mesh_terms: D000081:Acetamides / Q000627:therapeutic use; D000818:Animals; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001549:Benzamides / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D015507:Drugs, Investigational; D065130:Endothelin A Receptor Antagonists; D065131:Endothelin B Receptor Antagonists; D019332:Endothelin-1 / Q000378:metabolism; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000378:metabolism / Q000627:therapeutic use; D006162:Guanylate Cyclase / Q000037:antagonists & inhibitors; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000378:metabolism*; D000068877:Imatinib Mesylate; D008090:Lisuride / Q000031:analogs & derivatives / Q000627:therapeutic use; D009569:Nitric Oxide / Q000378:metabolism; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011719:Pyrazines / Q000627:therapeutic use; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D012702:Serotonin Antagonists / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use
chemical_list: D000081:Acetamides; D000959:Antihypertensive Agents; D001549:Benzamides; D015507:Drugs, Investigational; D065130:Endothelin A Receptor Antagonists; D065131:Endothelin B Receptor Antagonists; D019332:Endothelin-1; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011719:Pyrazines; D011720:Pyrazoles; D011743:Pyrimidines; D012702:Serotonin Antagonists; D013449:Sulfonamides; C006208:dironyl; D009569:Nitric Oxide; C048081:beraprost; C523468:selexipag; D000068877:Imatinib Mesylate; D011464:Epoprostenol; D008090:Lisuride; D006162:Guanylate Cyclase; C542595:riociguat; C427248:treprostinil; C533860:macitentan