Cardiopulmonary Exercise Testing Provides Prognostic Information in Advanced Cystic Fibrosis Lung Disease.
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Radtke Thomas, Urquhart Don S, Braun Julia, Barry Peter J, Waller Ian, Petch Nicole, Mei-Zahav Meir, Kramer Mordechai R, Hua-Huy Thong, Dinh-Xuan Anh Tuan, Innes J Alastair, McArthur Sara, Sovtic Aleksandar, Gojsina Bojana, Verges Samuel, de Maat Tanguy, Morrison Lisa, Wood Jamie, Crute Samantha, Williams Craig A, Tomlinson Owen W, Bar-Yoseph Ronen, Hebestreit Alexandra, Quon Bradley S, Kwong Eugenie, Saynor Zoe L, Causer Adam J, Stephenson Anne L, Schneiderman Jane E, Shaw Michelle, Dwyer Tiffany, Stevens Daniel, Remus Natascha, Douvry Benoit, Foster Karla, Benden Christian, Ratjen Felix, Hebestreit Helge, Prognostic Value of CPET in CF Study Group
Аннотация
Rationale: Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for patients with advanced CF lung disease 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 1 second (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 modeled 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 percentage predicted peak oxygen uptake ([Formula: see text]o2peak) and peak work rate (Wpeak) as significant predictors of death/LTX: adjusted hazard ratios per each additional 10% 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 11 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 advanced CF lung disease, and Wpeak appears to be a promising marker for LTX referral and candidate selection.