Autor: |
Gallotti R; Department of Pediatric Cardiology, Mattel Children's Hospital UCLA, 200 UCLA Medical Plaza, Suite 224, Los Angeles, CA, 90095, USA., Drogalis-Kim DE; Department of Pediatric Cardiology, Mattel Children's Hospital UCLA, 200 UCLA Medical Plaza, Suite 224, Los Angeles, CA, 90095, USA., Satou G; Department of Pediatric Cardiology, Mattel Children's Hospital UCLA, 200 UCLA Medical Plaza, Suite 224, Los Angeles, CA, 90095, USA.; Division of Pulmonary Hypertension, Mattel Children's Hospital UCLA, Los Angeles, CA, 90095, USA., Alejos J; Department of Pediatric Cardiology, Mattel Children's Hospital UCLA, 200 UCLA Medical Plaza, Suite 224, Los Angeles, CA, 90095, USA. jalejos@mednet.ucla.edu.; Division of Pulmonary Hypertension, Mattel Children's Hospital UCLA, Los Angeles, CA, 90095, USA. jalejos@mednet.ucla.edu. |
Abstrakt: |
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. |