title: Pulmonary arterial hypertension: a review in pharmacotherapy.
abstract: Pulmonary arterial hypertension (PAH) is a progressive disease that remains incurable. The past 2 decades have witnessed many advances in PAH-directed therapies. More recently, 3 new oral agents have become available in the United States within the past 2 years. Treprostinil is now available in extended-release oral tablets. Macitentan is the third endothelin receptor antagonist approved for use, demonstrating benefits on morbidity and mortality among patients with PAH in an event-driven study. Riociguat is the first soluble guanylate cyclase stimulator that has been approved for use in the United States. This article reviews the clinical efficacy and safety of these 3 agents and the roles they play in the management of PAH. Additionally, we review the limitations of using surrogate markers such as change in 6-minute walk distance to assess disease progression.
authors.affiliation: From the Department of Pharmacy, Montefiore Medical Center, Bronx, NY.
authors.forename: Bhaumik B
authors.initials: BB
authors.lastname: Patel
authors.forename: Ying
authors.initials: Y
authors.lastname: Feng
authors.forename: Angela
authors.initials: A
authors.lastname: Cheng-Lai
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D018450:Disease Progression; D004358:Drug Therapy / Q000639:trends*; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology*; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome; D016138:Walking / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents; D011720:Pyrazoles; D011743:Pyrimidines; D013449:Sulfonamides; D011464:Epoprostenol; C542595:riociguat; C427248:treprostinil; C533860:macitentan