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

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

Research output: Contribution to journalArticle

17 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)383-385
Number of pages3
JournalPediatric Pulmonology
Volume41
Issue number4
DOIs
StatePublished - Apr 2006

Fingerprint

Exercise Tolerance
Pulmonary Hypertension
Cystic Fibrosis
Lung Diseases
Exercise
Vascular Diseases
Lung
Sildenafil Citrate
Therapeutics

Keywords

  • 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

Cite this

Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease. / Montgomery, Gregory; Sagel, Scott D.; Taylor, Amy L.; Abman, Steven H.

In: Pediatric Pulmonology, Vol. 41, No. 4, 04.2006, p. 383-385.

Research output: Contribution to journalArticle

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