title: Pulmonary hypertension in idiopathic pulmonary fibrosis with mild-to-moderate restriction.
abstract: The clinical course of pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) is not known except in advanced disease.488 subjects in a placebo-controlled study of ambrisentan in IPF with mild-moderate restriction in lung volume, underwent right heart catheterisation (RHC) at baseline and 117 subjects (24%) had repeated haemodynamic measurements at 48 weeks. The subjects were categorised into a) World Health Organization (WHO) Group 3 PH (PH associated with pulmonary disease), n=68 (14%); b) WHO Group 2 PH (PH associated with left-sided cardiac disease), n=25 (5%); c) no PH but elevated pulmonary artery wedge pressure (PAWP), n=21 (4%); and d) no PH but without elevation of PAWP, n=374 (77%). The WHO Group 3 PH subjects had a lower diffusion capacity, 6MWD and oxygen saturation compared to the subjects with no PH. There was no significant change in mean pulmonary arterial pressure with ambrisenten or placebo after 12 months. Subjects with IPF associated with WHO Group 3 PH had impaired gas exchange and exercise capacity compared to patients without PH. An additional 9% of the subjects had haemodynamic evidence of subclinical left-ventricular dysfunction. Pulmonary artery pressures remained stable over 1 year in the majority of the cohort.
authors.affiliation: Dept of Medicine, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA, USA graghu@uw.edu.
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
authors.affiliation: Lung Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Nathan
authors.affiliation: Dept of Internal Medicine V, Comprehensive Pneumology Center, and Asklepios-Clinics-Gauting, Member of the German Center for Lung Research, Germany.
authors.forename: Juergen
authors.initials: J
authors.lastname: Behr
authors.affiliation: Dept of Medicine, National Jewish Health, Denver, CO, USA.
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.affiliation: Dept of Pulmonary Medicine, Mater Misericordiae Hospital, Dublin, Ireland.
authors.forename: James J
authors.initials: JJ
authors.lastname: Egan
authors.affiliation: Pulmonary Vascular Disease Program, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
authors.affiliation: Dept of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Kevin R
authors.initials: KR
authors.lastname: Flaherty
authors.affiliation: Dept of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
authors.affiliation: Interstitial Lung Disease, Royal Brompton Hospital, London, UK.
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
authors.affiliation: Dept of Biostatistics, Gilead Sciences Inc., Seattle, WA, USA.
authors.forename: Lixin
authors.initials: L
authors.lastname: Shao
authors.affiliation: Dept of Biostatistics, Gilead Sciences Inc., Seattle, WA, USA.
authors.forename: Huafeng
authors.initials: H
authors.lastname: Zhou
authors.affiliation: Dept of Medical Affairs, Gilead Sciences Inc., Foster City, CA, USA.
authors.forename: Noreen
authors.initials: N
authors.lastname: Henig
authors.affiliation: Dept of Clinical Research, Gilead Sciences Inc., Foster City, CA, USA.
authors.forename: Javier
authors.initials: J
authors.lastname: Szwarcberg
authors.affiliation: Dept of Clinical Research, Gilead Sciences Inc., Foster City, CA, USA.
authors.forename: Hunter
authors.initials: H
authors.lastname: Gillies
authors.affiliation: Dept of Clinical Research, Cardeas Pharma, Seattle, WA, USA.
authors.forename: Alan B
authors.initials: AB
authors.lastname: Montgomery
authors.affiliation: Dept of Clinical Research, Gilead Sciences Inc., Seattle, WA, USA.
authors.forename: Thomas G
authors.initials: TG
authors.lastname: O'Riordan
mesh_terms: D000368:Aged; D062186:Arterial Pressure; D006328:Cardiac Catheterization; D004311:Double-Blind Method; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology*; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications* / Q000188:drug therapy; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010666:Phenylpropionates / Q000008:administration & dosage*; D011669:Pulmonary Wedge Pressure / Q000187:drug effects*; D011724:Pyridazines / Q000008:administration & dosage*; D018487:Ventricular Dysfunction, Left; D014944:World Health Organization
chemical_list: D010666:Phenylpropionates; D011724:Pyridazines; C467894:ambrisentan