Pulmonary arterial hypertension: a review in pharmacotherapy

Cardiol Rev. 2015 Jan-Feb;23(1):33-51. doi: 10.1097/CRD.0000000000000042.

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.

Publication types

  • Review

MeSH terms

  • Antihypertensive Agents / therapeutic use*
  • Disease Progression*
  • Drug Therapy / trends*
  • Epoprostenol / analogs & derivatives
  • Epoprostenol / therapeutic use
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology*
  • Pyrazoles / therapeutic use
  • Pyrimidines / therapeutic use
  • Sulfonamides / therapeutic use
  • Time Factors
  • Treatment Outcome
  • Walking / physiology

Substances

  • Antihypertensive Agents
  • Pyrazoles
  • Pyrimidines
  • Sulfonamides
  • Epoprostenol
  • riociguat
  • treprostinil
  • macitentan