WS01.01 Cardiopulmonary exercise testing provides prognostic information in advanced cystic fibrosis lung disease

T. Radtke, D. S. Urquhart, J. Braun, P. Barry, I. Waller, N. Petch, M. Mei-Zahav, M. R. Kramer, T. Hua-Huy, A. T. Dinh-Xuan, J. A. Innes, S. Mcarthur, A. Sovtic, B. Gojsina, S. Verges, T. De Maat, L. Morrison, J. Wood, S. Crute, C. A. WilliamsO. W. Tomlinson, R. Bar-Yoseph, A. Hebestreit, B. S. Quon, E. Kwong, Z. L. Saynor, A. J. Causer, A. l. Stephenson, J. E. Schneiderman, M. Shaw, T. Dwyer, D. Stevens, N. Remus, B. Douvry, K. Foster, F. Ratjen, C. Benden, H. Hebestreit

Research output: Contribution to journalMeeting Abstractpeer-review


Objectives: Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for people with advanced CF lung disease (ACFLD) is currently unknown. We aimed to determine the prognostic value of CPET on the risk of death or lung transplant (LTX) within 2-years.

Methods: We retrospectively collected data from 20 CF-centers in Asia, Australia, Europe, and North America on adults with CF with a forced expiratory volume in 1 s (FEV1) ≤40% predicted who had performed a cycle ergometer CPET between January 2008 and December 2017. Time to death/LTX within 2-years after CPET was analysed using mixed Cox proportional hazards regression. Conditional inference trees were modelled to identify subgroups with an increased risk of death/LTX.

Results: In total, 174 patients (41.4% female) with mean±SD age of 30.0 ± 9.2 years and FEV1 30.9 ± 5.8% predicted were included. Forty-four patients (25.5%) died or underwent LTX within a mean ± SD follow-up period of 311 ± 191 days. Cox regression analysis adjusted forage, sex, and FEV1, revealed percent predicted peak oxygen uptake (VO2peak) and peak work rate (Wpeak) as significant predictors of death/LTX: adjusted hazard ratios per each additional ten more percent predicted were 0.60 (95% confidence interval [CI]: 0.43–0.90, P-value 0.008) and 0.60 (95% CI: 0.48–0.82, P-value <0.001), respectively. Tree-structured regression models, including a set of twelve prognostic factors for survival in CF, identified Wpeak to be most strongly associated with 2-year risk of death/LTX. Probability of death/LTX was 45.2% for those with a Wpeak ≤49.2% predicted versus 10.9% for those with a Wpeak >49.2% predicted (P <0.001).

Conclusion: CPET provides prognostic information in ACFLD and Wpeak appears to be a promising marker for aiding clinical decision-making with regard to referral for and timing of LTX.
Original languageEnglish
Pages (from-to)S1
Number of pages1
JournalJournal of Cystic Fibrosis
Issue numberSupplement 2
Publication statusPublished - 9 Jun 2023
Event46th European Cystic Fibrosis Conference - Vienna, Austria
Duration: 7 Jun 200210 Jun 2023

Cite this