Macitentan in the Treatment of Pulmonary Arterial Hypertension

Abstract

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:

• Eisenmenger syndrome
• endothelin receptor
• endothelin receptor antagonists
• ERA
• macitentan
• macitentan trials Scleroderma
• PAH specific therapies
• portoPH
• pulmonary hypertension

Financial & competing interests disclosure

T Pulido has served on advisory boards from Actelion/Janssen, Bayer, GSK and Pfizer. His institution has received research grants from Actelion/Janssen, Bayer, REATA and United Therapeutics. He has paid lectures from Actelion/Janssen, Bayer and Pfizer. R Zebadúa has paid lectures from Actelion/Janssen and Bayer. N Zayas has served in advisory boards from Actelion/Janssen, Bayer and Pfizer and has paid lectures from Actelion/Janssen and Bayer. J López has received paid lectures from Bayer. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Company review disclaimer

In addition to the peer-review process, with the author’s consent, the manufacturer of the product discussed in this article was given the opportunity to review the manuscript for factual accuracy. Changes were made by the author at their discretion and based on scientific or editorial merit only. The author maintained full control over the manuscript, including content, wording and conclusions.