Helge Hebestreit1, Erik H J Hulzebos2, Jane E Schneiderman3, Chantal Karila4, Steven R Boas5, Susi Kriemler6, Tiffany Dwyer7,8,9, Margareta Sahlberg10, Don S Urquhart11, Larry C Lands12, Felix Ratjen3, Tim Takken2, Liobou Varanistkaya1, Viktoria Rücker13, Alexandra Hebestreit1, Jakob Usemann14, Thomas Radtke6. 1. 1 University Children's Hospital, Wuerzburg, Germany. 2. 2 Child Development & Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands. 3. 3 Division of Respiratory Medicine, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada. 4. 4 Service de Pneumologie et Allergologie Pédiatriques, Centre de Ressources et Compétences dans la Mucoviscidose, Hôpital Necker Enfants Malades, Université Paris V - Descartes, Paris, France. 5. 5 Northwestern University Feinberg School of Medicine, Chicago, Illinois. 6. 6 Epidemiology, Biostatistics and Prevention Institute, University of Zurich, Zurich, Switzerland. 7. 7 Discipline of Physiotherapy, Faculty of Health Sciences and. 8. 9 Central Clinical School, Sydney Medical School, University of Sydney, Sydney, Australia. 9. 8 Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, Australia. 10. 10 Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Goteborg, Sweden. 11. 11 Department of Paediatric Respiratory and Sleep Medicine, Royal Hospital for Sick Children, Edinburgh, United Kingdom. 12. 12 Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada. 13. 13 Institute for Clinical Epidemiology and Biometry, Wuerzburg, Germany; and. 14. 14 University Children's Hospital Basel, Basel, Switzerland.
Abstract
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 ( V. o2peak) 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 V. o2peak 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.
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 ( V. o2peak) 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 V. o2peak 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.
Authors: Amanda P Silva; Erika V Araujo; Igor P Machado; Livia F Alves; Marcos F DA Silva Mello; Pedro Henrique DE A Silva; Isabella R Dias; Viviane Soares Journal: Int J Exerc Sci Date: 2021-04-01
Authors: Narelle S Cox; Beverley Eldridge; Sarah Rawlings; Julianna Dreger; Jennifer Corda; Jennifer Hauser; Brenda M Button; Jennifer Bishop; Amanda Nichols; Anna Middleton; Nathan Ward; Tiffany Dwyer; Owen W Tomlinson; Sarah Denford; Alan R Barker; Craig A Williams; Michael Kingsley; Paul O'Halloran; Anne E Holland Journal: BMC Pulm Med Date: 2019-12-19 Impact factor: 3.317
Authors: Bradley S Quon; Andrew H Ramsook; Satvir S Dhillon; Reid A Mitchell; Kyle G Boyle; Pearce G Wilcox; Jordan A Guenette Journal: Respir Res Date: 2020-06-01
Authors: M Marcella Burghard; G Gitte Berkers; S Sophie Ghijsen; Fm Francis Hollander-Kraaijeveld; Km Karin de Winter-de Groot; Ck Kors van der Ent; Hgm Harry Heijerman; T Tim Takken; Hj Erik Hulzebos Journal: Pediatr Pulmonol Date: 2020-03-31