Abstract
This retrospective chart review of patients less than 18 years old with pulmonary arterial hypertension (PAH) receiving selexipag was conducted to describe selexipag dosing practices, impact on concomitant PAH therapies, and the safety and efficacy of selexipag. Twenty-seven patients aged 1–17 years started a median dose of oral selexipag 100 µg twice daily. Therapy was increased by a median of 100 µg twice daily every 6 days to a maximally tolerated median dose of 800 µg twice daily. All 24 patients on another prostacyclin derivative were able to discontinue therapy at their maximum tolerated selexipag dose; other concomitant PAH therapies did not change. Changes in echocardiogram data and 6-MWT results were variable. No patients discontinued selexipag; four patients received decreased doses due to flushing (n = 1), drug interactions (n = 2), or increased frequency of nose bleeds (n = 1).
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No datasets were generated or analysed during the current study.
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All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by MG. The first draft of the manuscript was written by MG and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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Ethical approval was waived by the local Ethics Committee of Children’s Hospital of Philadelphia in view of the retrospective nature of the study and all the procedures being performed were part of the routine care.
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Grossman, M., Walker, S. & Ramsey, E.Z. Selexipag Dosing Strategies for Pediatric Patients with Pulmonary Arterial Hypertension. Pediatr Cardiol (2024). https://doi.org/10.1007/s00246-024-03513-w
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DOI: https://doi.org/10.1007/s00246-024-03513-w