Pulmonary Arterial Hypertension

Heart Fail Clin. 2018 Jul;14(3):255-269. doi: 10.1016/j.hfc.2018.02.003.


This article provides an overview of pulmonary arterial hypertension (PAH), beginning with the initial pathologic recognition of pulmonary hypertension more than 100 years ago and progressing to the current diagnostic categorization of PAH. It reviews the epidemiology, pathophysiology, genetics, and modern treatment of PAH. The article discusses several important recent studies that have highlighted the importance of new management strategies, including serial risk assessment and combination pharmacotherapy.

Keywords: BMPR2; Endothelin receptor antagonists; Phosphodiesterase-5 inhibitors; Prostacyclin; Pulmonary arterial hypertension; Pulmonary hypertension; REVEAL Registry.

Publication types

  • Review

MeSH terms

  • Antihypertensive Agents / therapeutic use*
  • Drug Therapy, Combination
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / physiopathology
  • Male
  • Molecular Targeted Therapy / methods
  • Risk Factors
  • Survival Rate


  • Antihypertensive Agents