EPS7.06 cardiovascular function in people with cystic fibrosis established on elexacaftor/tezacaftor/ivacaftor modulator therapy

Clayton, L., Shepherd, A., Corbett, J., Perissiou, Maria, Connett, G., Legg, J., Allenby, M., Daniels, T., Urquhart, D., Mackintosh, K., McNarry, M. and Saynor, Z. (2024) EPS7.06 cardiovascular function in people with cystic fibrosis established on elexacaftor/tezacaftor/ivacaftor modulator therapy. Journal of Cystic Fibrosis, 23 (Suppl. 1), S54-S55. (doi:10.1016/S1569-1993(24)00275-3).

Abstract

Background: people with cystic fibrosis (CF) are at increased risk of cardiovascular disease (CVD). Risk factors include CF-related diabetes, high dietary intake of fat and salt, and chronic inflammation associated with lung disease. An increased body mass index (BMI) and lowphysical activity (PA) may also contribute.

Methods: we will present data from ‘Understand-CF’, a cross-sectional study investigating various physiological responses in people with CF established on Elexacaftor/Tezacaftor/Ivacaftor (ETI). Specifically, we assessed: resting pulmonary function, BMI, macrovascular endothelial function (via flow-mediated dilation (FMD) of the brachial artery), microvascular endothelial function (via transdermal delivery of acetylcholine (ACh) and insulin (INS)) on the forearm), blood pressure, cardiac output, stroke volume and heart rate during ramp incremental cycling cardiopulmonary exercise testing (CPET) (via thoracic impedance cardiography), CPET-derived aerobic fitness and accelerometer-based indices of PA and sleep.

Results: data will be presented for these variables, comparing 15 pwCF (age: 29.7 ± 19.9 y; FEV1: 85.7 ± 23.0%predicted; BMI: 22.6 ± 3.4 kg/m2) and 15 healthy age- and sex-matched controls (age: 30.5 ± 20.0 y; FEV1: 91.1 ± 9.8%; BMI: 22.9 ± 5.4 kg/m2). Parameters will include peak oxygen consumption, time spent being sedentary and doing light- and moderate-to-vigorous PA, sleep duration and efficiency, blood pressure, %FMD change from baseline, and ACh and INS maximum cutaneous vascular conductance and area under the curve.

Conclusion: in the era of CFTR modulator therapy, understanding the prevalence and factors underpinning cardiovascular manifestations in people with CF is increasingly important. The present study will, for the first time, provide a comprehensive cross-sectional insight into micro- and macrovascular function alongside other important CVD risk factors in people with CF on ETI.

Funding: Cystic Fibrosis Warriors charity (No 1178063) grant.

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Contributors

Author: Z. Saynor

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