Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease |
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Authors: | Montgomery Gregory S Sagel Scott D Taylor Amy L Abman Steven H |
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Affiliation: | Division of Pediatric Pulmonology, Department of Pediatrics, Indiana University Medical Center, Indianapolis, Indiana, USA. gsmontgo@iupui.edu |
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Abstract: | Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy. |
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Keywords: | cystic fibrosis exercise pulmonary hypertension sildenafil 6‐min walk test phosphodiesterase inhibitor echocardiogram |
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