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Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis‐related lung disease
Author(s) -
Montgomery Gregory S.,
Sagel Scott D.,
Taylor Amy L.,
Abman Steven H.
Publication year - 2006
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20393
Subject(s) - medicine , sildenafil , cystic fibrosis , pulmonary hypertension , exercise intolerance , respiratory disease , pulmonary function testing , restrictive lung disease , lung , cardiology , lung disease , disease , pulmonary fibrosis , heart failure
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. Pediatr Pulmonol. © 2006 Wiley‐Liss, Inc.