title: Treating pulmonary hypertension in pediatrics.
abstract: BACKGROUND
  Pulmonary hypertension is a hemodynamic condition occurring rarely in pediatrics. Nevertheless, it is associated with significant morbidity and mortality. When characterized by progressive pulmonary vascular structural changes, the disease is called pulmonary arterial hypertension (PAH). It results in increased pulmonary vascular resistance and eventual right ventricular failure. In the vast majority of cases, pediatric PAH is idiopathic or associated with congenital heart disease, and, contrary to adult PAH, is rarely associated with connective tissue, portal hypertension, HIV infection or thromboembolic disease.
  
  
  METHODS
  This article reviews the current drug therapies available for the management of pediatric PAH. These treatments target the recognized pathophysiological pathways of PAH with endothelin-1 receptor antagonists, prostacyclin analogs and PDE type 5 inhibitors. New treatments and explored pathways are briefly discussed.
  
  
  CONCLUSIONS
  Although there is still no cure for PAH, quality of life and survival have been improved significantly with specific drug therapies. Nevertheless, management of pediatric PAH remains challenging, and depends mainly on results from adult clinical trials and pediatric experts. Further research on PAH-specific treatments in the pediatric population and data from international registries are needed to identify optimal therapeutic strategies and treatment goals in the pediatric population.
authors: Hôpitaux Universitaires de Genève, Département des Spécialités de Médecine, Service de Pneumologie, Programme Hypertension Pulmonaire , Geneva , Switzerland.
authors: Frédéric
authors: F
authors: Lador
authors: Nicole
authors: N
authors: Sekarski
authors: Maurice
authors: M
authors: Beghetti
mesh_terms: D002648:Child; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D015658:HIV Infections; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000188:drug therapy*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011788:Quality of Life
chemical_list: D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D011464:Epoprostenol
keywords: pediatrics; pulmonary hypertension; registries; strategy; treatment goals; treatments