• Helge Hebestreit, Hulzebos Erik H J EHJ 2 Child Development & Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands., Jane E Schneiderman, Chantal Karila, Steven R Boas, Susi Kriemler, Tiffany Dwyer, Margareta Sahlberg, Don S Urquhart, Larry C Lands, Felix Ratjen, Tim Takken, Liobou Varanistkaya, Viktoria Rücker, Alexandra Hebestreit, Jakob Usemann, Thomas Radtke, and Prognostic Value of CPET in CF Study Group.
• 1 University Children's Hospital, Wuerzburg, Germany.
• Am. J. Respir. Crit. Care Med. 2019 Apr 15; 199 (8): 987-995.

RationaleThe 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.ObjectivesTo determine the prognostic value of CPET-derived measures beyond peak oxygen uptake ( V . o2peak) following rigorous adjustment for other predictors.MethodsData 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 ResultsCox 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.ConclusionsCPET 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.

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