Selexipag Dosing Strategies for Pediatric Patients with Pulmonary Arterial Hypertension

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Madeline Grossman1, Stephen Walker2, E Zachary Ramsey3

  • 1Department of Pharmacy Services, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA. grossmanm3@chop.edu.

Pediatric Cardiology
|May 10, 2024
PubMed

Insights

Selexipag effectively treats pediatric pulmonary arterial hypertension (PAH), allowing discontinuation of other prostacyclin therapies. Dosing was escalated to a median of 800 µg twice daily with manageable side effects.

Area of Science:

  • Pediatric Cardiology
  • Pharmacology

Background:

  • Pulmonary arterial hypertension (PAH) is a severe condition affecting children.
  • Selexipag, a prostacyclin pathway enhancer, shows promise for PAH treatment.

Purpose of the Study:

  • To evaluate selexipag dosing, efficacy, and safety in pediatric PAH patients.
  • To assess the impact of selexipag on concomitant PAH therapies.

Main Methods:

  • Retrospective chart review of pediatric patients (<18 years) with PAH treated with selexipag.
  • Analysis of selexipag dosing titration, concomitant medications, and adverse events.

Main Results:

  • Median starting dose was 100 µg twice daily, escalated to a median maximally tolerated dose of 800 µg twice daily.
  • All patients on prostacyclin derivatives discontinued them while on selexipag.
  • Echocardiogram and 6-minute walk test (6-MWT) results showed variable changes.
  • Four patients required dose reduction due to flushing, drug interactions, or epistaxis; no patients discontinued selexipag.

Conclusions:

  • Selexipag is a viable treatment option for pediatric PAH, facilitating the cessation of other prostacyclin therapies.
  • Selexipag demonstrated a manageable safety profile in this pediatric cohort.

Keywords:
PediatricsProstacyclin derivativeProstacyclin receptor agonistPulmonary hypertensionSelexipag

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