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Cystic Fibrosis (CF), the most common inherited disease in Caucasians, is characterized by chronic pulmonary inflammation and progressive loss of gas exchange units that eventually results in respiratory failure. There is strong evidence that, in CF, abnormally low perfusion carries a high risk of death independent from the presence of pulmonary hypertension. However, the evolution of pulmonary vascular disease in CF and how it might contribute to the rate of decline in lung function is not known. Our knowledge remains limited to the results of old observational studies which concluded that the major causes of pulmonary vascular remodeling and hypertension in CF are hypoxic respiratory failure and destruction of lung tissue. Our recent data obtained by state-of-the-art Magnetic Resonance Imaging (MRI) of the pulmonary circulation, challenges the existing paradigm. We demonstrate that in the absence of hypoxia, significant changes in pulmonary perfusion and in surrogate measures of vascular resistance as well as in collateral blood flow begin early in the course of CF. Newly developed therapeutics have altered dramatically the course of patients suffering from pulmonary vascular disease. Through this 8 week trial, we will examine by Magnetic Resonance Imaging the effect of Sildenafil on pulmonary perfusion and systemic vascularization of the lungs in subjects with mild to moderate disease.
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Inclusion and exclusion criteria
Inclusion Criteria
Research subjects must meet the following inclusion criteria:
Exclusion Criteria
Research subjects will be excluded from the study based on:
History of CF-related liver disease with portal hypertension
Currently smoking cigarettes or other tobacco products
Use of daytime oxygen supplementation
Previous organ transplantation
Unstable or uncontrolled hypertension
Ongoing use of oral corticosteroids
For female subjects: pregnancy or lactation and unwillingness to use contraception during study participation
Any hemodynamically significant congenital or acquired cardiac disease or significant cardiomyopathy, hematologic disease (i.e. hemoglobinopathies), or pulmonary disease associated with an increased risk of pulmonary perfusion defects or pulmonary hypertension other than as an outcome of CF
History of renal and/or hepatic insufficiency, defined as cystatin-C level that exceeds normal range and a previous diagnosis of liver cirrhosis.
History of uncontrolled asthma defined as oral steroid dependent
History of hypersensitivity to gadolinium (Magnevist)
Contraindications specific to MRI including a history of claustrophobia, cardiac pacemaker, or other non-MRI compatible surgical implants (This includes neuro-stimulators containing electrical circuitry, or which generate electrical signals and/or have moving metal parts, and metal orthopedic pins or plates. The research coordinator and/or the MRI technologist will screen all subjects using the standard checklist of medical history and safety questions used by the Radiology Department in routine clinical scans.)
Daily use of montelukast and ibuprofen
Use of nitrate medicines or other drugs known to have unsafe interactions with Sildenafil
Known allergy to Sildenafil
Inability to comply with study procedures
History of the following:
Laboratory Exclusion Criteria for research subjects (based on history or blood work before first MRI):
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Data sourced from clinicaltrials.gov
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