Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil

University of California, Los Angeles

Overview

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.

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.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

QUADRUPLE

Conditions

Pulmonary Arterial Hypertension Idiopathic Pulmonary Fibrosis Interstitial Lung Disease Pulmonary Hypertension

Interventions

sildenafilDRUG

the dose of sildenafil will be 20mg three times per day (orally)

Eligibility

Sex: ALL

Medical Language ↔ Plain English

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

Locations (1)

David Geffen School of Medicine UCLA

Los Angeles, California, United States

Outcomes

Primary Outcomes

6 minute walk distance (6MWD) change from Baseline

ATS guideline based 6MW distance

Time frame: 6 months

Secondary Outcomes

Right heart catheterization hemodynamics

pulmonary hemodynamics via invasive right heart catheterization

Time frame: 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

Chemokine analysis on peripheral blood

evaluation of a group of chemokines before and after the intervention in each arm

Time frame: the chemokines will be quantified and compared between study entry and the time point just prior to lung transplantation

Quality of life assessment

SF-36 (short-form 36)

Time frame: 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

Quality of life assessment in the context of dyspnea

Saint George Respiratory Questionnaire (SGRQ)

Time frame: 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

Data from ClinicalTrials.gov

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