title: Advances in pediatric pulmonary arterial hypertension.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality in children. Approved medications for the treatment of adult PAH have been used to treat children, but evidence-based treatment algorithms for children are lacking.
  
  
  RESULTS
  Pediatric PAH registries have begun to define the incidence and prevalence of idiopathic PAH and PAH associated with congenital heart disease. A pediatric-specific classification of pulmonary hypertensive vascular disease has been proposed. Furthermore, the first randomized placebo-controlled trial of type-5 phosphodiesterase therapy in treatment-naïve children with PAH has been completed and reported. This trial highlights the importance of the difficulties of performing clinical trials in children with targeted PAH therapy as well as the importance of long-term follow-up of adverse events.
  
  
  CONCLUSIONS
  Classification, clinical trials, and therapy for children with PAH must take into account the unique aspects of PAH in children.
authors.affiliation: The Children's Hospital Heart Institute, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA. dunbar.ivy@childrenscolorado.org
authors.forename: Dunbar
authors.initials: D
authors.lastname: Ivy
mesh_terms: D002648:Child; D065627:Familial Primary Pulmonary Hypertension; D014943:Global Health; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology; D009017:Morbidity / Q000639:trends; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D015996:Survival Rate / Q000639:trends
chemical_list: D058986:Phosphodiesterase 5 Inhibitors