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).
Similar content being viewed by others
Data Availability
No datasets were generated or analysed during the current study.
References
Hansmann G, Koestenberger M, Alastalo TP et al (2019) 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: the European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant 38(9):879–901. https://doi.org/10.1016/j.healun.2019.06.022
Kim JS, McSweeney J, Lee J, Ivy D (2016) Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: pharmacotherapies in cardiac critical care pulmonary hypertension. Pediatr Crit Care Med 17(3 Suppl 1):S89–S100. https://doi.org/10.1097/PCC.0000000000000622
Gorenflo M, Ziesenitz VC (2021) Treatment of pulmonary arterial hypertension in children. Cardiovasc Diagn Ther 11(4):1144–1159. https://doi.org/10.21037/cdt-20-912
Remodulin® (treprostinil) Injection, for subcutaneous or intravenous use [package insert]. Research Triangle Park: Unite Therapeutics Corp.; 2002
Veletri (epoprostenol) for Injection [package insert]. South San Francisco, CA: Actelion Pharmaceuticals US, Inc.; 1995
Flolan® (epoprostenol) for Injection [package insert]. Research Triangle Park: GlaxoSmithKline; 2008
Ventavis® (iloprost) inhalation solution, for oral inhalation use [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; 2004
Orenitram® (treprostinil) extended-release tablets, for oral use [package insert]. Research Triangle Park: Unite Therapeutics Corp.; 2002
Uptravi® (selexipag) [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; 2022
Gavotto A, Thomas F, Werner O et al (2020) Use of treprostinil in pediatric pulmonary hypertension: case reports and review of the literature. J Cardiovasc Pharmacol 76(1):23–31. https://doi.org/10.1097/FJC.0000000000000820
Tapson VF, Torres F, Kermeen F et al (2012) Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial. Chest 142(6):1383–1390. https://doi.org/10.1378/chest.11-2212
Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galiè N, Ghofrani H-A, Hoeper MM, Lang IN, Preiss R, Rubin LJ, Di Scala L, Tapson V, Adzerikho I, Liu J, Moiseeva O, Zeng X, Simonneau G, McLaughlin VV, GRIPHON Investigators (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373(26):2522–2533. https://doi.org/10.1056/NEJMoa1503184
McSweeney J, Colglazier E, Becerra J et al (2023) Failure to tolerate continuous subcutaneous treprostinil in pediatric pulmonary hypertension patients. Pulm Circ 13(2):e12224. https://doi.org/10.1002/pul2.12224
Hansmann G, Meinel K, Bukova M, Chouvarine P, Wåhlander H, Koestenberger M, European Pediatric Pulmonary Vascular Disease Network (EPPVDN) (2020) Selexipag for the treatment of children with pulmonary arterial hypertension: first multicenter experience in drug safety and efficacy. J Heart Lung Transplant 39(7):695–706. https://doi.org/10.1016/j.healun.2020.03.029
Gallotti R, Drogalis-Kim DE, Satou G, Alejos J (2017) Single-center experience using selexipag in a pediatric population. Pediatr Cardiol 38(7):1405–1409. https://doi.org/10.1007/s00246-017-1677-7
Lafuente-Romero A, Rodriguez OA (2021) Selexipag use for paediatric pulmonary hypertension: a single centre report focussed on congenital heart disease patients. Cardiol Young 31(9):1513–1515. https://doi.org/10.1017/S1047951121000937
Youssef D, Richards S, Lague S et al (2023) A Canadian, retrospective, multicenter experience with selexipag for a heterogeneous group of pediatric pulmonary hypertension patients. Front Pediatr 28(11):1055158. https://doi.org/10.3389/fped.2023.1055158
Colglazier E, Stevens L, Parker C et al (2022) Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension. Pulm Circ 12(4):e12159. https://doi.org/10.1002/pul2.12159
Author information
Authors and Affiliations
Contributions
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.
Corresponding author
Ethics declarations
Competing interests
The authors have no relevant financial or non-financial interests to disclose.
Ethical Approval
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.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
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
Received:
Accepted:
Published:
DOI: https://doi.org/10.1007/s00246-024-03513-w