{Reference Type}: Journal Article
{Title}: Cardiopulmonary Exercise Testing Provides Prognostic Information in Advanced Cystic Fibrosis Lung Disease.
{Author}: Radtke T;Urquhart DS;Braun J;Barry PJ;Waller I;Petch N;Mei-Zahav M;Kramer MR;Hua-Huy T;Dinh-Xuan AT;Innes JA;McArthur S;Sovtic A;Gojsina B;Verges S;de Maat T;Morrison L;Wood J;Crute S;Williams CA;Tomlinson OW;Bar-Yoseph R;Hebestreit A;Quon BS;Kwong E;Saynor ZL;Causer AJ;Stephenson AL;Schneiderman JE;Shaw M;Dwyer T;Stevens D;Remus N;Douvry B;Foster K;Benden C;Ratjen F;Hebestreit H; ;
{Journal}: Ann Am Thorac Soc
{Volume}: 21
{Issue}: 3
{Year}: 2024 Mar 25
{Factor}: 8.785
{DOI}: 10.1513/AnnalsATS.202304-317OC
{Abstract}: 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.