Division of Chronic Disease Epidemiology, Epidemiology, Biostatistics and Prevention Institute, and.
Department of Paediatric Respiratory and Sleep Medicine, Royal Hospital for Children and Young People, Edinburgh, United Kingdom.
Ann Am Thorac Soc. 2024 Mar;21(3):411-420. doi: 10.1513/AnnalsATS.202304-317OC.
Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for patients with advanced CF lung disease is unknown. To determine the prognostic value of CPET on the risk of death or lung transplant (LTX) within 2 years. 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 (FEV) ⩽ 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. In total, 174 patients (FEV, 30.9% ± 5.8% predicted) were included. Forty-four patients (25.5%) died or underwent LTX. Cox regression analysis adjusted for age, sex, and FEV revealed percentage predicted peak oxygen uptake ([Formula: see text]o) and peak work rate (W) as significant predictors of death/LTX: adjusted hazard ratios per each additional 10% predicted were 0.60 (95% confidence interval, 0.43-0.90; = 0.008) and 0.60 (0.48-0.82; < 0.001). Tree-structured regression models, including a set of 11 prognostic factors for survival, identified W to be most strongly associated with 2-year risk of death/LTX. Probability of death/LTX was 45.2% for those with a W ⩽ 49.2% predicted versus 10.9% for those with a W > 49.2% predicted ( < 0.001). CPET provides prognostic information in advanced CF lung disease, and W appears to be a promising marker for LTX referral and candidate selection.
心肺运动测试（CPET）可提供囊性纤维化（CF）的预后信息；然而，其在 CF 晚期肺病患者中的预后价值尚不清楚。本研究旨在确定 CPET 在 2 年内死亡或肺移植（LTX）风险中的预后价值。我们回顾性收集了 2008 年 1 月至 2017 年 12 月期间在亚洲、澳大利亚、欧洲和北美 20 个 CF 中心接受过 1 秒用力呼气量（FEV）≤40%预计值的患者进行的踏车 CPET 数据。使用混合 Cox 比例风险回归分析死亡/LTX 的时间。使用条件推断树对死亡/LTX 风险增加的亚组进行建模。共纳入 174 例患者（FEV，30.9%±5.8%预计值）。44 例（25.5%）患者死亡或接受 LTX。调整年龄、性别和 FEV 的 Cox 回归分析显示，预计峰值摄氧量百分比（[Formula: see text]o）和峰值功（W）是死亡/LTX 的显著预测因子：每增加 10%预计值，调整后的风险比分别为 0.60（95%置信区间，0.43-0.90； = 0.008）和 0.60（0.48-0.82； < 0.001）。包括 11 个生存预后因素的树状结构回归模型确定 W 与 2 年死亡/LTX 风险的关联最强。W ≤ 49.2%预计值的患者死亡/LTX 的概率为 45.2%，而 W > 49.2%预计值的患者为 10.9%（ < 0.001）。CPET 可提供 CF 晚期肺病的预后信息，而 W 似乎是 LTX 转诊和候选者选择的有前途的标志物。
