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.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
NONE
62.5mg orally bid for first month, followed by 125mg bid thereafter
David Geffen School of Medicine UCLA
Los Angeles, California, United States
Departments of Pulmonary and Critical Care, Cardiothoracic Surgery and Infectious Diseases at David Geffen School of Medicine at UCLA
Los Angeles, California, United States
6 minute walk distance
ATS Guideline 6MW distance before and after intervention
Time frame: monthly assessement until date of lung transplantation
right heart catheterization hemodynamics
pulmonary hemodynamics
Time frame: variable based on time between listing and actual lung transplantation
chemokine peripheral blood analysis
battery of chemokines analyzed from the peripheral blood
Time frame: monthly
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