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

Study Overview
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Brief Summary
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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.

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

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

Conditions
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Pulmonary Arterial Hypertension, Idiopathic Pulmonary Fibrosis

Intervention / Treatment
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* Drug: bosentan


Participation Criteria
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Eligibility Criteria
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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
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: treatment with bosentan<br>patients with resting or exercise induced PAH receive bosentan in a randomized open label fashion | Drug: bosentan<br>* 62.5mg orally bid for first month, followed by 125mg bid thereafter<br>* Other names: tracleer;|
| No Intervention: PAH group with no therapy<br>patients with resting or exercise PAH get randomized to receive no specific therapy | |
| No Intervention: No PAH and no therapy<br>patients with no evidence of either resting or exercise PAH receive no intervention but are followed until lung transplantation | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 6 minute walk distance | ATS Guideline 6MW distance before and after intervention | monthly assessement until date of lung transplantation | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| right heart catheterization hemodynamics | pulmonary hemodynamics | variable based on time between listing and actual lung transplantation | 
| chemokine peripheral blood analysis | battery of chemokines analyzed from the peripheral blood | monthly | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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bosentan, pulmonary arterial hypertension, idiopathic pulmonary fibrosis