RT Journal Article
SR Electronic
T1 Pulmonary hypertension in idiopathic pulmonary fibrosis with mild-to-moderate restriction
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1370
OP 1377
DO 10.1183/13993003.01537-2014
VO 46
IS 5
A1 Ganesh Raghu
A1 Steven D. Nathan
A1 Juergen Behr
A1 Kevin K. Brown
A1 James J. Egan
A1 Steven M. Kawut
A1 Kevin R. Flaherty
A1 Fernando J. Martinez
A1 Athol U. Wells
A1 Lixin Shao
A1 Huafeng Zhou
A1 Noreen Henig
A1 Javier Szwarcberg
A1 Hunter Gillies
A1 Alan B. Montgomery
A1 Thomas G. O'Riordan
YR 2015
UL http://erj.ersjournals.com/content/46/5/1370.abstract
AB 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.In IPF associated with pulmonary hypertension (PH), gas exchange is severely impaired; the PH was stable over 1 year http://ow.ly/OnvHl