title: Pulmonary arterial hypertension in children: a medical update.
abstract: Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by a progressive pulmonary vasculopathy with ensuing right heart failure if left untreated. In the 1980's, prior to the current treatment era, idiopathic pulmonary arterial hypertension (IPAH) carried a poor prognosis with a 10 month median survival for children after diagnosis. However, in 1995 continuous intravenous epoprostenol was approved for the treatment of severe PAH, improving hemodynamics, quality of life, exercise capacity, functional class and survival. In the past decade there have been further advances in the treatment of PAH; however, there is still no cure. While much of the groundbreaking clinical research has been performed in adults, children have also seen the benefits of PAH novel therapies. The target population among pediatric patients is expanding with the recent recognition of pulmonary hypertension as a risk factor for sickle cell disease patients. With rapid advances, navigating the literature becomes challenging. A comprehensive review of the most recent literature over the past year on available and emerging novel therapies as well as an approach to target pediatric populations provides insights into the management of pediatric PAH patients.
authors.affiliation: Department of Pediatrics, Columbia University College of Physician & Surgeons, New York, NY 10032, USA. esb14@columbia.edu
authors.forename: Erika B
authors.initials: EB
authors.lastname: Rosenzweig
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
mesh_terms: D000755:Anemia, Sickle Cell / Q000453:epidemiology; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002648:Child; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology*; D016285:Iloprost / Q000627:therapeutic use; D007262:Infusions, Intravenous; D010666:Phenylpropionates / Q000627:therapeutic use; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D011724:Pyridazines / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D010666:Phenylpropionates; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors; D011724:Pyridazines; D011464:Epoprostenol; C467894:ambrisentan; D016285:Iloprost