Idiopathic Pulmonary Fibrosis, Interstitial Lung Disease, Pulmonary Arterial Hypertension, Pulmonary Hypertension
Conditions
Keywords
pulmonary hypertension, pulmonary arterial hypertension, interstitial lung disease, idiopathic pulmonary fibrosis
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 sildenafil may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.
Detailed 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.
Interventions
DRUGsildenafil
the dose of sildenafil will be 20mg three times per day (orally)
Sponsors
Pfizer
University of California, Los Angeles
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
QUADRUPLE (Subject, Caregiver, Investigator, Outcomes Assessor)
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 6 minute walk distance (6MWD) change from Baseline | 6 months | ATS guideline based 6MW distance |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Right heart catheterization hemodynamics | initial right heart catheterization compared to catheterization done on day of lung transplantation; we will specifically compare mean pulmonary artery pressure, pulmonary vascular resistance, and pulmonary artery wedge pressure | pulmonary hemodynamics via invasive right heart catheterization |
| Chemokine analysis on peripheral blood | the chemokines will be quantified and compared between study entry and the time point just prior to lung transplantation | evaluation of a group of chemokines before and after the intervention in each arm |
| Quality of life assessment | study entry compared to 6 months or at the time of lung transplantation (whichever comes first); there is a 0-100 scoring scale and the lower the score, the more disability | SF-36 (short-form 36) |
| Quality of life assessment in the context of dyspnea | the SGRQ will be be compared at two time points, study entry and at 6 months or the time of transplantation (whichever comes first); scores range from 0 to 100 and higher scores indicate more limitation | Saint George Respiratory Questionnaire (SGRQ) |
Countries
United States
Outcome results
None listed