Novel medical therapies for pulmonary arterial hypertension

Clin Chest Med. 2013 Dec;34(4):867-80. doi: 10.1016/j.ccm.2013.08.002.


Available targeted therapies for pulmonary arterial hypertension are capable only of slowing progression of the disease and a cure remains elusive. However with the improved understanding of the pulmonary vascular remodeling that characterizes the disease, there is optimism that the disconnect between preclinical and clinical studies may be bridged with some of the newer therapies that are now at different stages of clinical evaluation. This article examines the evidence behind these new candidate treatments that may become part of the arsenal available for clinicians managing this devastating disease.

Trial registration: NCT00469027.

Keywords: Endothelial progenitor cells; Endothelin receptor antagonists; Guanylate cyclase stimulators; Prostacyclin receptor agonists; Rho-kinase inhibitors; Tyrosine kinase inhibitors.

Publication types

  • Review

MeSH terms

  • Endothelin Receptor Antagonists*
  • Familial Primary Pulmonary Hypertension
  • Guanylate Cyclase / antagonists & inhibitors*
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Pyrazoles / therapeutic use*
  • Pyrimidines / therapeutic use*
  • Receptors, Epoprostenol / agonists*
  • Sulfonamides / therapeutic use*


  • Endothelin Receptor Antagonists
  • Pyrazoles
  • Pyrimidines
  • Receptors, Epoprostenol
  • Sulfonamides
  • Guanylate Cyclase
  • riociguat
  • macitentan

Associated data