Publication date: Jul 01, 2025
Maintaining good cardiopulmonary fitness is crucial for global health in cystic fibrosis (CF), but there is limited knowledge about the longitudinal evolution of fitness measures at young ages, particularly with recent therapeutic advances. We investigated trends in exercise test outcomes in young people with CF and how they are influenced by elexacaftor, tezacaftor and ivacaftor (ETI) and the coronavirus disease 2019 (COVID-19) pandemic. 70 young people with CF (12-20 years old) were included in this retrospective observational single-centre study. We reviewed 177 symptom-limited surveillance cardiopulmonary exercise tests (CPETs) conducted on a cycle ergometer between 2010 and 2024. Longitudinal trends in peak oxygen uptake (V’ ), maximal workload and ventilation were analysed using linear mixed models, separately for periods before and after the COVID-19 pandemic onset and ETI introduction. In ETI-naive people with CF, V’ was lower, and the increase from 12 to 20 years was smaller than predicted by validated reference data (p
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| Concepts | Keywords |
|---|---|
| 20years | Cardiopulmonary |
| Coronavirus | Cf |
| Fitness | Covid |
| Paediatric | Cystic |
| Retrospective | Eti |
| Exercise | |
| Fibrosis | |
| Fitness | |
| Limited | |
| Long | |
| Longitudinal | |
| Pandemic | |
| Term | |
| Trends | |
| Young |
Semantics
| Type | Source | Name |
|---|---|---|
| disease | MESH | cystic fibrosis |
| disease | MESH | coronavirus disease 2019 |
| disease | IDO | symptom |
| drug | DRUGBANK | Oxygen |