Medical treatment update on pulmonary arterial hypertension

Cher Y. Enderby, Charles Burger

Research output: Contribution to journalReview articlepeer-review

12 Scopus citations


Pulmonary arterial hypertension is a chronic, progressive disease of the pulmonary vasculature resulting in poor outcomes if left untreated. The management of group 1 pulmonary arterial hypertension has included the use of prostanoids, phosphodiesterase-5 inhibitors, and endothelin receptor antagonists targeting the prostacyclin, endothelin-1, and nitric oxide pathways. Three new medications have been approved by the US Food and Drug Administration over the past couple of years. Macitentan is the newest endothelin receptor antagonist, riociguat is a soluble guanylate cyclase stimulator, and treprostinil diolamine is the first oral prostanoid. This review will focus on the key trials leading to their approval, special considerations for each medication, and their potential place in therapy. The use of combination therapy as initial therapy in pulmonary arterial hypertension will also be discussed.

Original languageEnglish (US)
Pages (from-to)264-272
Number of pages9
JournalTherapeutic Advances in Chronic Disease
Issue number5
StatePublished - Sep 2015


  • Macitentan
  • Oral treprostinil
  • Pulmonary arterial hypertension
  • Riociguat

ASJC Scopus subject areas

  • Medicine (miscellaneous)


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