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

Thomas Radtke, Don S. Urquhart, Julia Braun, Peter J. Barry, Ian Waller, Nicole Petch, Meir Mei-Zahav, Mordechai R. Kramer, Thong Hua-Huy, Anh Tuan Dinh-Xuan, J. Alastair Innes, Sara McArthur, Aleksandar Sovtic, Bojana Gojsina, Samuel Verges, Tanguy De Maat, Lisa Morrison, Jamie Wood, Samantha Crute, Craig A. WilliamsOwen W. Tomlinson, Ronen Bar-Yoseph, Alexandra Hebestreit, Bradley S. Quon, Eugenie Kwong, Zoe L. Saynor, Adam J. Causer, Anne L. Stephenson, Jane E. Schneiderman, Michelle Shaw, Tiffany Dwyer, Daniel Stevens, Natascha Remus, Benoit Douvry, Karla Foster, Christian Benden, Felix Ratjen, Helge Hebestreit

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Rationale: Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for patients with advanced CF lung disease (ACFLD) is unknown.

Objectives: 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 patients with a forced expiratory volume in 1s (FEV1) ≤40% predicted who performed a cycle ergometer CPET between January 2008 and December 2017. Time to death/LTX was analyzed using mixed Cox proportional hazards regression. Conditional inference trees were modelled to identify subgroups with increased risk of death/LTX.

Results: In total, 174 patients (FEV1 30.9±5.8% predicted) were included. Forty-four patients (25.5%) died or underwent LTX. Cox regression analysis adjusted for age, sex and FEV1, revealed percent predicted peak oxygen uptake (V ̇O2peak) and peak work rate (Wpeak) as significant predictors of death/LTX: adjusted hazard ratios per each additional ten percent predicted were 0.60 (95% confidence interval, 0.43–0.90, P=0.008) and 0.60 (0.48–0.82, P<0.001). Tree-structured regression models, including a set of twelve prognostic factors for survival, 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.

Conclusions: CPET provides prognostic information in ACFLD and Wpeak appears to be a promising marker for LTX referral and candidate selection.
Original languageEnglish
JournalAnnals of the American Thoracic Society
Early online date25 Oct 2023
Publication statusEarly online - 25 Oct 2023

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