Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease

Gregory S. Montgomery, Scott D. Sagel, Amy L. Taylor, Steven H. Abman

19 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)383-385
Number of pages3
JournalPediatric pulmonology
Issue number4
StatePublished - Apr 1 2006



  • 6-min walk test
  • Cystic fibrosis
  • Echocardiogram
  • Exercise
  • Phosphodiesterase inhibitor
  • Pulmonary hypertension
  • Sildenafil

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

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