Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan



Status:Withdrawn
Conditions:High Blood Pressure (Hypertension), Pulmonary
Therapuetic Areas:Cardiology / Vascular Diseases, Pulmonary / Respiratory Diseases
Healthy:No
Age Range:Any
Updated:4/17/2018
Start Date:October 2007
End Date:December 2009

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Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study

Pulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is
a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently
there is no significant data to support the use of pulmonary vasodilators for PAH in the
setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH
either at rest or during exercise. The study hypothesis is that bosentan may improve
morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or
exercise PAH.

The purpose of this study was to evaluate bosentan in the setting of exercise or resting
pulmonary hypertension in patients with underlying pulmonary fibrosis.

Inclusion Criteria:

- Idiopathic Pulmonary Fibrosis referred for lung transplantation

- Minimum 50 meter 6 minute walk distance

- No significant underlying liver disease

Exclusion Criteria:

- Significant liver disease or cirrhosis

- non ambulatory

- previous adverse reaction/allergy to Bosentan
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Los Angeles, CA
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10833 Le Conte Ave
Los Angeles, California 90095
(310) 825-4321
David Geffen School of Medicine, UCLA In 2002 Mr. David Geffen announced a $200 million...
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Los Angeles, CA
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