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Exercise induced pulmonary hypertension in systemic sclerosis and treatment with ambrisentan: A prospective single center, open label, pilot study.
MedLine Citation:
PMID:  22777623     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Exercise-induced pulmonary hypertension (ePH) may represent an early, clinically relevant phase in the spectrum of pulmonary vascular disease. The purpose of this pilot study was to describe the changes in hemodynamics and exercise capacity in patients with SSc-spectrum associated ePH treated with open-label daily ambrisentan (AMB). METHODS: SSc-spectrum patients exercised using a supine lower extremity cycle ergometer at baseline and 24 weeks. All patients had normal hemodynamics at rest. Baseline ePH required: mean pulmonary artery pressure (mPA) > 30mmHg with maximum exercise and transpulmonary gradient (TPG) > 15mmHg. Patients were given either 5mg or 10mg of AMB once daily for 24 weeks. Primary endpoint was change in exercise pulmonary vascular resistance (PVR). Secondary endpoints included an improvement from baseline in 6MWD, health-related quality of life assessments, and cardiopulmonary hemodynamics. RESULTS: 11 of 12 enrolled patients completed study. At 24 weeks, mean exercise PVR (85.8 dyn/s/cm5, p=0.003) and mean 6MW (44.5 m, p=0.0007) improved. Improvements were also observed in: mean exercise CO (1.4 L/min, p=0.006), mean mPA (-4.1mmHg, p=0.02), total pulmonary resistance (TPR) (-93.0 dyn/s/cm5, p=0.0008). Three patients developed resting PAH during the 24 weeks. CONCLUSIONS: Exercise hemodynamics and exercise capacity in patients with SSc-spectrum associated exercise PH improved over 24 weeks with exposure to AMB. Placebo-controlled studies are needed to confirm whether this is a drug-related effect and to determine optimal therapeutic regimens for patients with exercise PH.
Authors:
R Saggar; D Khanna; S Shapiro; D E Furst; P Maranian; P Clements; F Abtin; Shiv Dua; J Belperio; R Saggar
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-7-6
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  -     ISSN:  1529-0131     ISO Abbreviation:  -     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-7-10     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 by the American College of Rheumatology.
Affiliation:
Thoracic Transplantation, Heart-Lung Institute, St. Joseph Hospital & Medical Center, Phoenix, AZ 85013.
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