Macitentan in the treatment of pulmonary arterial hypertension

Future Cardiol. 2021 Jan;17(1):49-58. doi: 10.2217/fca-2020-0012. Epub 2020 Jul 17.


Pulmonary arterial hypertension (PAH) is an uncommon but lethal and progressive disease in which prostacyclin, nitric oxide and endothelin-1 pathways are disturbed and contribute to the pathophysiology of this disease. Endothelin receptor antagonists are a class of drugs that have been approved as PAH therapy. Macitentan is a lipophilic, tissue specific, dual receptor antagonist with a higher potency than bosentan and a reduced risk of hepatic injury. Macitentan has shown a reduction in morbidity and mortality due to PAH at long-term follow-up and improvements in hemodynamics, exercise capacity and functional class at the short term. Its main adverse events are nasopharyngitis, bronchitis and an increased risk of anemia. We review the clinical data of macitentan and its use in PAH.

Keywords: ERA; Eisenmenger syndrome; PAH specific therapies; endothelin receptor; endothelin receptor antagonists; macitentan; macitentan trials Scleroderma; portoPH; pulmonary hypertension.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Humans
  • Hypertension, Pulmonary* / drug therapy
  • Pulmonary Arterial Hypertension*
  • Pyrimidines / therapeutic use
  • Sulfonamides / therapeutic use


  • Pyrimidines
  • Sulfonamides
  • macitentan