The use of Selexipag in managing Pediatric Pulmonary Hypertension

A recent study discusses the safety and effectiveness of selexipag for treating pediatric patients with pulmonary arterial hypertension (PAH).

After analyzing clinical parameters before selexipag, 3-12 months after selexipag, and >12 months follow-up, the study observed:

• Inclusion of 87 patients.
• Selexipag administration as an add-on to background therapy in 32 participants.
• The transition from another prostanoid in 55. 
• 4.7 and 28.5 μg/kg/dose twice daily, as the median starting and final doses, respectively. 
• Improved indexed pulmonary to systemic vascular resistance ratio in add-on patients after selexipag initiation with a lower average mean pulmonary artery pressure and oxygen consumption. 
• Stable mean pulmonary artery pressure and a lower mean indexed pulmonary vascular resistance but late functional worsening in some Transition patients with VO2 max decreased at follow-up. 
• Side effects in 40% of participants.
• Treatment discontinuation in only 2% of participants.

This study proves the tolerability and effectiveness of the oral prostacyclin agonist selexipag. Add-on patients have early hemodynamic improvement, while transition patients have early stability with a risk of late functional worsening, highlighting the importance of ongoing monitoring.

Frank BS, Gentzler ER, Avitabile CM, et al. Safety and Effectiveness of Selexipag in Pediatric Pulmonary Hypertension: A Retrospective Multicenter Cohort Study. The Journal of Pediatrics. 2024;275