Prioritising cardiopulmonary exercise testing for adults with cystic fibrosis: a service evaluation.

McDowell R; University of Bath, Bath, UK.
Ogbonnaya C; Shannon H; Douglas H

BMC Pulmonary Medicine. 26(1), 2026 Feb 19.

BACKGROUND: Cystic Fibrosis is an inherited, life-limiting condition
causing a range of symptoms including lowered exercise tolerance.
Approximately 95% of people with cystic fibrosis in the United Kingdom are
now eligible for new genetic modulator therapies. As a result, cystic
fibrosis centres are treating older populations in greater numbers.
Cardiopulmonary exercise testing measures aerobic capacity, however it is
resource intensive. Identifying whether routinely collected clinical
measures are associated with reduced aerobic capacity is needed to aid
prioritisation of cardiopulmonary exercise testing.
METHODS: Maximal
cardiopulmonary exercise testing data were collected from July 2022 to
January 2024, alongside routine clinical data (spirometry, body mass
index, diabetic status, Pseudomonas aeruginosa colonisation status,
modulator status, age and sex). Peak oxygen uptake was analysed as a
percentage predicted value (VO2peakpp).
RESULTS: Overall aerobic capacity
at the centre was low (mean peak oxygen uptake 79.16% predicted). No
relationship was identified between body mass index and aerobic capacity
(beta = 0.23, 95%CI -0.91, 1.37, p = 0.69). When adjusting for other
clinical measures, having cystic fibrosis related diabetes (beta=-17.56,
95%CI -27.17, -7.95, p < 0.001) and younger age (beta = 16.62, 95%CI 4.13,
29.12, p = 0.01) were associated with a reduction in VO2peakpp.
CONCLUSION
: Annual CPET for all pwCF may not be necessary or available. This service
evaluation found associations with younger age and CFRD and reduced
VO2peak who could be targeted