Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil

University of California, Los Angeles (UCLA)

Status and phase: Withdrawn; Phase 4

Conditions: Pulmonary Hypertension; Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease; Pulmonary Arterial Hypertension

Treatments: Drug: sildenafil

Study type: Interventional

Funder types: Other; Industry

Identifiers

NCT00625079

IPF/PH

Details and patient eligibility

About

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 sildenafil may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.

Full description

The purpose of this study is to evaluate the use of sildenafil in patients with pulmonary fibrosis and PH being considered for lung transplantation. We hypothesize that not only will sildenafil improve functionality and QOL in the pre-transplant setting but it may also improve primary graft dysfunction after lung transplantation.

Sex: All

Volunteers: No Healthy Volunteers

Inclusion criteria

• Patients with Idiopathic Pulmonary Fibrosis referred for lung transplantation at our medical center
• Minimal 6 minute walk distance of 50 meters; must be able to conduct supine exercise during heart catheterization

Exclusion criteria

• Non ambulatory
• Prior adverse reaction/allergy to sildenafil or other PDE-5 Inhibitors
• Any other pulmonary vasodilator within one month of enrollment

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: Quadruple Blind

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0 participants in 3 patient groups, including a placebo group

Pre-transplant placebo

Placebo Comparator group

Description:

There are two placebo comparators.... one for the group of patients with resting PAH and another for the group of patients with exercise PAH

Treatment:

Drug: sildenafil

Pre-transplant sildenafil

Experimental group

Description:

There are two active comparators, one group with resting PAH and another with exercise PAH, both receiving drug.

Treatment:

Drug: sildenafil

Pre-transplant no PAH-specific therapy

No Intervention group

Description:

this group of patients has no evidence for either resting or exercise PAH but will be followed without specific drug intervention