Treatment

14 Consensus Statements for PAH Treatment Are Developed

An expert panel has developed 14 consensus statements that provide guidance on the initiation of oral prostacyclin pathway agents (PPAs) among adults with pulmonary arterial hypertension (PAH).

“The paucity of clinical evidence overall, and particularly from randomized trials in this setting, creates a gap in knowledge,” the panel wrote. “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.”

To develop its statements, the panel conducted a systematic literature search of studies published on MEDLINE between October 1, 2008, and October 1, 2018.

The panel considered idiopathic, heritable, repaired congenital heart defect, and drug- or toxin-induced PAH as one etiologic grouping (IPAH+).

“The process was focused on the use of oral treprostinil or selexipag in patients with IPAH+ or connective tissue disease (CTD)-associated PAH and (functional class) FC II or III symptoms receiving background dual endothelin receptor antagonist (ERA)/phosphodiesterase type 5 inhibitor (PDE5i) therapy,” according to the panel.

The panel identified 13 clinical scenarios in which selexipag may be considered a treatment option, including for patients with IPAH+ who are receiving dual oral therapy with an ERA and PDE5i and who meet any of the following clinical scenario criteria: 

  • Patients with FC II symptoms and low-risk hemodynamic parameters and who have been hospitalized for PAH in the last 6 months, regardless of their right ventricular (RV) function, brain natriuretic peptide (BNP)/N-terminal prohormone of BNP (NT-proBNP) levels, or 6-minute walk distance (6MWD)
  • Patients with FC II symptoms and intermediate-risk hemodynamic parameters, regardless of hospitalization for PAH in the last 6 months, their RV function, BNP/NT-proBNP levels, or 6MWD
  • Patients with FC III symptoms and low-risk hemodynamic parameters, regardless of hospitalization for PAH in the last 6 months, their RV function, BNP/NT-proBNP levels, or 6MWD
  • Patients with FC III symptoms and intermediate-risk hemodynamic parameters who have not been hospitalized for PAH in the last 6 months, regardless of their RV function, BNP/NT-proBNP levels, or 6MWD

 

According to the panel, the use of selexipag may also be considered for patients with CTD-associated PAH and one of the following clinical scenarios:

  • Patients with FC II symptoms and low-risk hemodynamic parameters who have not been hospitalized for PAH in the last 6 months but who have any degree of RV dysfunction and abnormal BNP/NT-proBNP levels, irrespective of 6MWD
  • Patients with FC III symptoms and low-risk hemodynamic parameters and who have been hospitalized for PAH in the last 6 months, irrespective of their RV function, BNP/NT-proBNP levels, or 6MWD

 

“Ultimately, these consensus statements were limited to use of oral selexipag because the panel did not support the use of oral treprostinil for the presented clinical scenarios, consistent with published phase 3 clinical trial data at the time of the meeting,” the authors explained.

Meanwhile, the panel suggested that intravenous or subcutaneous prostacyclin is the treatment of choice for patients with IPAH+ or CTD-associated PAH who are receiving dual oral ERA/PDE5i therapy and who have high-risk hemodynamic parameters.

—Colleen Murphy

Reference:

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. Chest. 2020;157(4):955-965. doi:10.1016/j.chest.2019.10.043