Results of an Expert Consensus Survey on the Treatment of Pulmonary Arterial Hypertension With Oral Prostacyclin Pathway Agents

Vallerie V. McLaughlin, Richard Channick, Teresa De Marco, Harrison W. Farber, Sean Gaine, Nazzareno Galié, Richard A. Krasuski, Ioana Preston, Rogerio Souza, J. Gerry Coghlan, Robert P. Frantz, Anna Hemnes, Nick H. Kim, Irene M. Lang, David Langleben, Mengtao Li, Olivier Sitbon, Victor Tapson, Adaani Frost

Research output: Contribution to journalReview articlepeer-review

16 Scopus citations


Background: Treatment of pulmonary arterial hypertension (PAH) has evolved substantially over the past two decades and varies according to etiology, functional class (FC), hemodynamic parameters, and other clinical factors. Current guidelines do not provide definitive recommendations regarding the use of oral prostacyclin pathway agents (PPAs) in PAH. To provide guidance on the use of these agents, an expert panel was convened to develop consensus statements for the initiation of oral PPAs in adults with PAH. Methods: A systematic literature search was conducted using MEDLINE. The established RAND/University of California Los Angeles appropriateness method, which incorporates the Delphi method and the nominal group technique, was used to create consensus statements. Idiopathic, heritable, repaired congenital heart defect, and drug- or toxin-induced PAH (IPAH+) was considered as one etiologic grouping. The process was focused on the use of oral treprostinil or selexipag in patients with IPAH+ or connective tissue disease-associated PAH and FC II or III symptoms receiving background dual endothelin receptor antagonist/phosphodiesterase type 5 inhibitor therapy. Results: The panel developed 14 consensus statements regarding the appropriate use of oral PPAs in the target population. The panel identified 13 clinical scenarios in which selexipag may be considered as a treatment option. Conclusions: The paucity of clinical evidence overall, and particularly from randomized trials in this setting, creates a gap in knowledge. These consensus statements are intended to aid physicians in navigating treatment options and using oral PPAs in the most appropriate manner in patients with PAH.

Original languageEnglish (US)
Pages (from-to)955-965
Number of pages11
Issue number4
StatePublished - Apr 2020


  • oral prostacyclin
  • oral treprostinil
  • prostacyclin pathway agent
  • pulmonary arterial hypertension
  • selexipag

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine


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