Hostname: page-component-586b7cd67f-dsjbd Total loading time: 0 Render date: 2024-11-22T13:09:53.470Z Has data issue: false hasContentIssue false

Selexipag use for paediatric pulmonary hypertension: a single centre report focussed on congenital heart disease patients

Published online by Cambridge University Press:  30 March 2021

Alvaro Lafuente-Romero
Affiliation:
Pediatric Cardiology Section, “Gregorio Marañon” General University Hospital, Madrid, Spain
Alejandro Rodriguez Ogando
Affiliation:
Pediatric Cardiology Section, “Gregorio Marañon” General University Hospital, Madrid, Spain

Abstract

Pulmonary hypertension is a rare and complex disease with poor prognosis. Paediatric cases are infrequent and usually associated with congenital heart disease. Management is problematical due to the limited therapy available and poor evidence of efficacy. Recently a new medication, selexipag (UptraviR), a prostacyclin receptor agonist, has been approved for the treatment of pulmonary artery hypertension in adults. We report our experience using selexipag in four paediatric patients with pulmonary hypertension associated with congenital heart disease.

Type
Brief Report
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Rosenzweig, EB, Abman, SH, Adatia, I, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J 2019; 53: 1801916. Published 2019 Jan 24. doi: 10.1183/13993003.01916-2018 CrossRefGoogle Scholar
Hansmann, G, Koestenberger, M, Alastalo, TP, et al. 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 2019; 38: 879901. doi: 10.1016/j.healun.2019.06.022 CrossRefGoogle Scholar
Sitbon, O, Channick, R, Chin, KM, et al. GRIPHON investigators. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 2015; 373: 25222533.CrossRefGoogle ScholarPubMed
Geerdink, LM, Bertram, H, Hansmann, G. First-in-child use of the oral selective prostacyclin IP receptor agonist selexipag in pulmonary arterial hypertension. Pulm Circ 2017; 7: 551554. doi: 10.1177/2045893217703369 CrossRefGoogle ScholarPubMed
Gallotti, R, Drogalis-Kim, DE, Satou, G, Alejos, J. Single-center experience using Selexipag in a pediatric population. Pediatr Cardiol 2017; 38: 14051409. doi: 10.1007/s00246-017-1677-7 CrossRefGoogle Scholar
del Cerro Marin, MJ, Sabate Rotes, A, Rodriguez Ogando, A, et al. Assessing pulmonary hypertensive vascular disease in childhood. Data from the Spanish registry. Am J Respir Crit Care Med 2014; 190: 14211429.CrossRefGoogle ScholarPubMed
Yung, D, Widlitz, AC, Rosenzweig, EB, et al. Outcomes in children with idiopathic pulmonary arterial hypertension. Circulation 2004; 110: 660665.CrossRefGoogle ScholarPubMed
Kitterman, N, Poms, A, Miller, DP et al. Bloodstream infections in patients with pulmonary arterial hypertension treated with intravenous prostanoids: insights from the REVEAL REGISTRY. Mayo Clin Proc 2012; 87: 825834.CrossRefGoogle ScholarPubMed
Koestenberger, M, Hansmann, G. Should we use the oral selective IP receptor agonist selexipag off-label in children with pulmonary arterial hypertension? Pulm Circ 2018; 8. https://doi.org/10.1177/2045894018793580 Google ScholarPubMed
Duggan, ST, Keam, SJ, Burness, CB. Selexipag: a review in pulmonary arterial hypertension. Am J Cardiovasc Drugs 2016; 17: 7380. doi: 10.1007/s40256-016-0209-9 CrossRefGoogle Scholar