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BMC Pulmonary Medicine

, 14:74

Infectious, Rare and Idiopathic Pulmonary Diseases


BackgroundAdult patients with cystic fibrosis CF frequently have reduced exercise tolerance, which is multifactorial but mainly due to bronchial obstruction. The aim of this retrospective analysis was to determine the mechanisms responsible for exercise intolerance in patients with mild-to-moderate or severe disease.

MethodsCardiopulmonary exercise testing with blood gas analysis at peak exercise was performed in 102 patients aged 28 ± 11 years: 48 patients had severe lung disease FEV1 < 50%, group 1 and 54 had mild-to-moderate lung disease FEV1 ≥ 50%, group 2. VO2 peak was measured and correlated with clinical, biological, and functional parameters.

ResultsVO2 peak for all patients was 25 ± 9 mL-kg-min 65 ± 21% of the predicted value and was < 84% of predicted in 82% of patients 100% of group 1, 65% of group 2. VO2 peak was correlated with body mass index, C-reactive protein, FEV1, FVC, RV, DLCO, VE-VCO2 peak, VD-VT, PaO2, PaCO2, PA-aO2, and breathing reserve. In multivariate analysis, FEV1 and overall hyperventilation during exercise were independent determinants of exercise capacity R = 0.67. FEV1 was the major significant predictor of VO2 peak impairment in group 1, accounting for 31% of VO2 peak alteration, whereas excessive overall hyperventilation reduced or absent breathing reserve and VE-VCO2 accounted for 41% of VO2 alteration in group 2.

ConclusionExercise limitation in adult patients with CF is largely dependent on FEV1 in patients with severe lung disease and on the magnitude of the ventilatory response to exercise in patients with mild-to-moderate lung disease.

KeywordsCystic fibrosis Cardiopulmonary exercise testing Pulmonary function Exercise Electronic supplementary materialThe online version of this article doi:10.1186-1471-2466-14-74 contains supplementary material, which is available to authorized users.

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Autor: Jean Pastré - Anne Prévotat - Catherine Tardif - Carole Langlois - Alain Duhamel - Benoit Wallaert


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