title: Advances in the management of pediatric pulmonary hypertension.
abstract: Pulmonary hypertension is a rare disease in neonates, infants, and children, and is associated with substantial morbidity and mortality. An adequate understanding of the controlling pathophysiologic mechanisms is lacking. Moreover, a minority of research is focused specifically on neonatal  and pediatric populations. Although therapeutic options have increased over the past several decades, they remain limited. In advanced pulmonary hypertension, progressive pulmonary vascular functional and structural changes ultimately cause increased pulmonary vascular impedance, right-ventricular  failure, and death. Management includes the prevention and/or treatment of active pulmonary vasoconstriction, the support of right-ventricle function, treatment of the underlying disease (if possible), and the promotion of regressive remodeling of structural pulmonary vascular changes. Most  currently available therapies augment or inhibit factors, or mediators of their downstream signaling cascades, that originate in the pulmonary vascular endothelium. These pathways include nitric-oxide/cyclic guanosine monophosphate (cGMP), prostacyclin, and endothelin-1. The ability to reverse  advanced structural changes remains an as yet unattained goal. This paper reviews the epidemiology, pathophysiology, current treatments, and emerging therapies related to neonatal and pediatric pulmonary hypertension.
authors.affiliation: Division of Pediatric Critical Care Medicine, University of California, San Francisco, California 94143-1346, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Oishi
authors.forename: Sanjeev A
authors.initials: SA
authors.lastname: Datar
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Fineman
mesh_terms: D000818:Animals; D000959:Antihypertensive Agents / Q000494:pharmacology / Q000627:therapeutic use; D000077300:Bosentan; D002648:Child; D002956:Citrulline / Q000494:pharmacology / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004730:Endothelium, Vascular / Q000503:physiopathology; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000503:physiopathology / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D009119:Muscle Contraction / Q000502:physiology; D047495:PPAR gamma / Q000494:pharmacology / Q000627:therapeutic use; D010547:Persistent Fetal Circulation Syndrome / Q000503:physiopathology / Q000628:therapy; D058986:Phosphodiesterase 5 Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use; D011453:Prostaglandins / Q000494:pharmacology / Q000627:therapeutic use; D011687:Purines / Q000494:pharmacology / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000494:pharmacology / Q000627:therapeutic use; D013450:Sulfones / Q000494:pharmacology / Q000627:therapeutic use; D014655:Vascular Resistance / Q000502:physiology; D014661:Vasoconstriction / Q000502:physiology; D016278:Ventricular Function, Right
chemical_list: D000959:Antihypertensive Agents; D047495:PPAR gamma; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011453:Prostaglandins; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D002956:Citrulline; D000068677:Sildenafil Citrate; D000077300:Bosentan