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- Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, and Juan Alejos.
- Department of Pediatric Cardiology, Mattel Children's Hospital UCLA, 200 UCLA Medical Plaza, Suite 224, Los Angeles, CA, 90095, USA.
- Pediatr Cardiol. 2017 Oct 1; 38 (7): 1405-1409.
AbstractPulmonary 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.
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