Pediatric Cardiology

, Volume 38, Issue 7, pp 1405–1409 | Cite as

Single-Center Experience Using Selexipag in a Pediatric Population

  • Roberto Gallotti
  • Diana E. Drogalis-Kim
  • Gary Satou
  • Juan Alejos
Original Article

Abstract

Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi®), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH.

Keywords

Pediatrics Pulmonary hypertension Remodulin® Selexipag Treprostinil Uptravi® 

Notes

Compliance with Ethical Standards

Conflict of interest

Juan Alejos MD has received a speaker honorarium from Actelion Pharmaceuticals.

References

  1. 1.
    Hoeper MM, Bogaard HJ, Condliffe R et al (2013) Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D42–D50CrossRefPubMedGoogle Scholar
  2. 2.
    Farber HW, Loscalzo J (2004) Pulmonary arterial hypertension. N Engl J Med 351:1655–1665CrossRefPubMedGoogle Scholar
  3. 3.
    Farber HW, Miller D, Poms AD et al (2015) Five-year outcomes of patients enrolled in the REVEAL registry. Chest 148:1043–1054CrossRefPubMedGoogle Scholar
  4. 4.
    Haworth SG, Hislop AA (2009) Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001–2006. Heart 95:312–317CrossRefPubMedGoogle Scholar
  5. 5.
    Kitterman N, Poms A, Miller DP et al (2012) Bloodstream infections in patients with pulmonary arterial hypertension treated with intravenous prostanoids: insights from the REVEAL REGISTRY®. Mayo Clin Proc 87(9):825–834CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Kallen AJ, Lederman E, Balaji A et al (2008) Bloodstream infections in patients given treatment with intravenous prostanoids. Infect Control Hosp Epidemiol 29(4):342–349CrossRefPubMedGoogle Scholar
  7. 7.
    Marr CR, McSweeney JE, Mullen MP, Kulik TJ (2015) Central venous line complications with chronic ambulatory infusion of prostacyclin analogues in pediatric patients with pulmonary arterial hypertension. Pulm Circ 5(2):322–326CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Drogalis-Kim D, Jefferies J, Wilmot I, Alejos J (2016) Right sided heart failure and pulmonary hypertension: new insights into disease mechanisms and treatment modalities. Prog Pediatr Cardiol 43:71–80CrossRefGoogle Scholar
  9. 9.
    Sharma K (2016) Selexipag for the treatment of pulmonary arterial hypertension. Expert Rev Respir Med 10(1):1–3CrossRefPubMedGoogle Scholar
  10. 10.
    Takatsuki S, Parker DK, Doran AK, Friesen RH, Ivy DD (2013) Acute pulmonary vasodilator testing with inhaled treprostinil in children with pulmonary arterial hypertension. Pediatr Cardiol 34(4):1006–1012CrossRefPubMedGoogle Scholar
  11. 11.
    Sitbon O, Channick R, Chin KM et al (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373(26):2522–2533CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  • Roberto Gallotti
    • 1
  • Diana E. Drogalis-Kim
    • 1
  • Gary Satou
    • 1
    • 2
  • Juan Alejos
    • 1
    • 2
  1. 1.Department of Pediatric CardiologyMattel Children’s Hospital UCLALos AngelesUSA
  2. 2.Division of Pulmonary HypertensionMattel Children’s Hospital UCLALos AngelesUSA

Personalised recommendations