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Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis

Journal article
Authors H. Hebestreit
E. H. J. Hulzebos
J. E. Schneiderman
C. Karila
S. R. Boas
S. Kriemler
T. Dwyer
Margareta E Sahlberg
D. S. Urquhart
L. C. Lands
F. Ratjen
T. Takken
L. Varanistkaya
V. Rücker
A. Hebestreit
J. Usemann
T. Radtke
Published in American Journal of Respiratory and Critical Care Medicine
Volume 199
Issue 8
Pages 987-995
ISSN 1073-449X
Publication year 2019
Published at Institute of Clinical Sciences, Department of Pediatrics
Pages 987-995
Language en
Keywords Cystic fibrosis, Exercise testing, Lung transplantation, Peak oxygen uptake, Prognosis, carbon dioxide, adult, Article, body mass, cardiopulmonary exercise test, diabetes mellitus, exercise test, female, follow up, forced expiratory volume, high risk patient, human, lung gas exchange, major clinical study, male, nutritional status, oxygen consumption, patient counseling, phenotype, population, prevalence, priority journal, proportional hazards model, Pseudomonas aeruginosa, retrospective study, survival, survival rate, survivor
Subject categories Respiratory Medicine and Allergy


Rationale: The prognostic value of cardiopulmonary exercise testing (CPET) for survival in cystic fibrosis (CF) in the context of current clinical management, when controlling for other known prognostic factors, is unclear. Objectives: To determine the prognostic value of CPET-derived measures beyond peak oxygen uptake (VO 2 peak) following rigorous adjustment for other predictors. Methods: Data from 10 CF centers in Australia, Europe, and North America were collected retrospectively. A total of 510 patients completed a cycle CPET between January 2000 and December 2007, of which 433 fulfilled the criteria for a maximal effort. Time to death/lung transplantation was analyzed using Cox proportional hazards regression. In addition, phenotyping using hierarchical Ward clustering was performed to characterize high-risk subgroups. Measurements and Main Results: Cox regression showed, even after adjustment for sex, FEV1% predicted, body mass index (z-score), age at CPET, Pseudomonas aeruginosa status, and CF-related diabetes as covariates in the model, that VO 2 peak in % predicted (hazard ratio [HR], 0.964; 95% confidence interval [CI], 0.944-0.986), peak work rate (% predicted; HR, 0.969; 95% CI, 0.951-0.988), ventilatory equivalent for oxygen (HR, 1.085; 95% CI, 1.041-1.132), and carbon dioxide (HR, 1.060; 95% CI, 1.007-1.115) (all P < 0.05) were significant predictors of death or lung transplantation at 10-year follow-up. Phenotyping revealed that CPET-derived measures were important for clustering. We identified a high-risk cluster characterized by poor lung function, nutritional status, and exercise capacity. Conclusions: CPET provides additional prognostic information to established predictors of death/lung transplantation in CF. High-risk patients may especially benefit from regular monitoring of exercise capacity and exercise counseling. Copyright © 2019 by the American Thoracic Society.

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