title: Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension.
abstract: Pulmonary hypertension complicates the course of many newborns with congenital diaphragmatic hernia. In the most severe cases, the fetal condition of markedly elevated pulmonary vascular resistance persists after birth and is associated with hypoxemic respiratory failure and severe disturbances in cardiac performance. Late pulmonary hypertension (weeks to months after birth) is increasingly recognized in this population, and chronic pulmonary vascular abnormalities (months to years after birth) are now being discovered. In this review, we will discuss the pathophysiology of acute, late, and chronic pulmonary hypertension in patients with congenital diaphragmatic hernia. We will also review the role of currently available pulmonary vasoactive drugs in the management of pulmonary hypertension in this population.
authors.affiliation: Department of Pediatrics, Division of Neonatology, The Children's Hospital and the University of Colorado School of Medicine, Denver, CO 80218-1088, USA. john.kinsella@uchsc.edu
authors.forename: John P
authors.initials: JP
authors.lastname: Kinsella
authors.forename: D Dunbar
authors.initials: DD
authors.lastname: Ivy
authors.forename: Steven H
authors.initials: SH
authors.lastname: Abman
mesh_terms: D000208:Acute Disease; D000280:Administration, Inhalation; D001993:Bronchodilator Agents / Q000008:administration & dosage*; D002908:Chronic Disease; D006548:Hernia, Diaphragmatic / Q000150:complications; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D007231:Infant, Newborn; D009569:Nitric Oxide / Q000008:administration & dosage*; D010547:Persistent Fetal Circulation Syndrome / Q000150:complications / Q000188:drug therapy
chemical_list: D001993:Bronchodilator Agents; D009569:Nitric Oxide