A new consensus survey of expert opinions was published regarding the use of oral prostacyclin pathway agents in patients with various forms of pulmonary arterial hypertension (PAH) who received dual endothelin receptor antagonist (ERA)/phosphodiesterase type 5 inhibitor (PDE5i) therapy. The opinions were published in CHEST.

Researchers used MEDLINE to perform a systematic literature review of studies between 2008 and 2018 comprising adult patients with connective tissue disease (CTD)-associated PAH and function class (FC) 2 or 3 idiopathic, heritable, repaired congenital heart defect, or drug- or toxin-induced PAH (the latter was considered one etiologic grouping, IPAH+) who were treated with oral prostacyclin pathway agents. Pivotal trials for oral treprostinil and selexipag were also included if they focused on IPAH+ or CTD-associated PAH and FC 1 or 2 symptoms with background ERA/PDE5i therapy.

A series of clinical scenarios centered on 1 or the 3 etiologic groups (IPAH, CTD-associated PAH, and portopulmonary hypertension) were presented to the panelists for the development of the consensus. To create the consensus statements, the panel used the RAND/University of California Los Angeles Appropriateness Method, which incorporates the Delphi method and the nominal group technique.

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A total of 458 case scenarios were accepted by Delphi 2. Based on the available data, the panelists agreed that selexipag may be considered for patients with IPAH+ and low- or intermediate-risk hemodynamic parameters who receive dual oral ERA/PDE5i therapy. This was specific to patients in FC 2 who had not been hospitalized for PAH in the past 6 months but had moderate to severe right ventricular dysfunction.

The panelists also agreed that intravenous or subcutaneous prostacyclin should be the treatment of choice in patients with IPAH+ or CTD-associated PAH who receive dual oral therapies and have high-risk hemodynamic parameters. Overall, a total of 14 consensus statements on the use of oral prostacyclin pathway agents that included 13 clinical scenarios where selexipag may be considered were discussed by the panelists.

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A limitation of the survey included the paucity of evidence (only 5 clinical trials) regarding the use of selexipag and oral treprostinil.

Because of these limitations, the authors concluded that the “expert opinions must be validated with rigorous prospective studies, and this document may serve as a template for future investigations.”

Disclosure: This clinical trial was supported by Actelion Pharmaceuticals. Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.


McLaughlin VV, Channick R, De Marco T, et al. Results of an expert consensus survey on the treatment of pulmonary arterial hypertension with oral prostacyclin pathway agents [published online November 16, 2019]. CHEST. doi:10.1016/j.chest.2019.10.043