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

Sponsor

Rajan Saggar (Other)

Overall Status

Withdrawn

CT.gov ID

NCT00625469

Collaborator

Actelion (Industry)

Enrollment

Locations

Arms

Anticipated Duration (Months)

Patients Per Site

Patients Per Site Per Month

Study Details

Study Description

Brief Summary

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.

Condition or Disease	Intervention/Treatment	Phase
Pulmonary Arterial Hypertension Idiopathic Pulmonary Fibrosis	Drug: bosentan	Phase 4

Detailed Description

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 Design

Study Type:

Interventional

Actual Enrollment :

0 participants

Allocation:

Randomized

Intervention Model:

Parallel Assignment

Masking:

None (Open Label)

Primary Purpose:

Treatment

Official Title:

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

Actual Study Start Date :

Oct 1, 2007

Anticipated Primary Completion Date :

Jun 1, 2009

Anticipated Study Completion Date :

Dec 1, 2009

Arms and Interventions

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

Arm

Intervention/Treatment

Experimental: treatment with bosentan

patients with resting or exercise induced PAH receive bosentan in a randomized open label fashion

Drug: bosentan

62.5mg orally bid for first month, followed by 125mg bid thereafter

Other Names:

tracleer

No Intervention: PAH group with no therapy

patients with resting or exercise PAH get randomized to receive no specific therapy

No Intervention: No PAH and no therapy

patients with no evidence of either resting or exercise PAH receive no intervention but are followed until lung transplantation

Outcome Measures

Primary Outcome Measures

6 minute walk distance [monthly assessement until date of lung transplantation]
ATS Guideline 6MW distance before and after intervention

Secondary Outcome Measures

right heart catheterization hemodynamics [variable based on time between listing and actual lung transplantation]
pulmonary hemodynamics
chemokine peripheral blood analysis [monthly]
battery of chemokines analyzed from the peripheral blood

Eligibility Criteria

Criteria

Ages Eligible for Study:

N/A and Older

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

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

Contacts and Locations

Locations

	Site	City	State	Country	Postal Code
1	David Geffen School of Medicine UCLA	Los Angeles	California	United States	90095-1690
2	Departments of Pulmonary and Critical Care, Cardiothoracic Surgery and Infectious Diseases at David Geffen School of Medicine at UCLA	Los Angeles	California	United States	90095-1690