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Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study

Lauren J Clayton Orcid Logo, Anthony I Shepherd Orcid Logo, Jo Corbett Orcid Logo, Maria Perissiou Orcid Logo, Gary Connett, Julian Legg, Mark Allenby Orcid Logo, Thomas Daniels Orcid Logo, Don S Urquhart Orcid Logo, Kelly Mackintosh Orcid Logo, Melitta McNarry Orcid Logo, Zoe L Saynor Orcid Logo

Journal of Cystic Fibrosis, Volume: 24, Issue: 4, Pages: 801 - 808

Swansea University Authors: Kelly Mackintosh Orcid Logo, Melitta McNarry Orcid Logo

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DOI (Published version): 10.1016/j.jcf.2025.02.001

Abstract

Background: Cystic fibrosis (CF) has been associated with impaired cardiovascular and endothelial function. CF transmembrane conductance regulator (CFTR) modulator therapy, most recently, Elexacaftor/Tezacaftor/Ivacaftor (ETI) has led to improved CFTR function and life expectancy, however, the risin...

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Published in: Journal of Cystic Fibrosis
ISSN: 1569-1993 1873-5010
Published: Elsevier BV 2025
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URI: https://cronfa.swan.ac.uk/Record/cronfa69150
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CF transmembrane conductance regulator (CFTR) modulator therapy, most recently, Elexacaftor/Tezacaftor/Ivacaftor (ETI) has led to improved CFTR function and life expectancy, however, the rising prevalence of obesity in adults is concerning. This study assessed the micro- and macro-vascular endothelial function, cardiovascular disease (CVD) risk factors, and physical activity (PA) profiles in people with CF (pwCF) on ETI compared to healthy matched controls. Methods: In 15 pwCF and 15 age- and sex-matched controls, microvascular endothelial function (via transdermal delivery of insulin [INS] and acetylcholine [ACh] on the forearm), macrovascular endothelial function (via flow-mediated dilation [FMD] of the brachial artery), central haemodynamic parameters, including heart rate (HR), stroke volume index (SVi) and cardiac output index (Q&#x307;I) (via thoracic impedance cardiography), body mass index (BMI), blood pressure (BP), and accelerometer-assessed PA, were measured. Results: There were no differences in INS or FMD-mediated vasodilation between the groups (P&gt;0.05). However, a reduced vasodilatory response was evident in pwCF following ACh-mediated vasodilation (P=0.01) and FMD normalised for shear rate (P=0.03). No differences in resting HR, SVi, Q&#x307;I, BP, BMI or PA were found (P&gt;0.05). Conclusion: This study demonstrated reduced micro- and macrovascular function in pwCF. 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spelling 2025-09-30T10:56:16.7464937 v2 69150 2025-03-25 Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study bdb20e3f31bcccf95c7bc116070c4214 0000-0003-0355-6357 Kelly Mackintosh Kelly Mackintosh true false 062f5697ff59f004bc8c713955988398 0000-0003-0813-7477 Melitta McNarry Melitta McNarry true false 2025-03-25 EAAS Background: Cystic fibrosis (CF) has been associated with impaired cardiovascular and endothelial function. CF transmembrane conductance regulator (CFTR) modulator therapy, most recently, Elexacaftor/Tezacaftor/Ivacaftor (ETI) has led to improved CFTR function and life expectancy, however, the rising prevalence of obesity in adults is concerning. This study assessed the micro- and macro-vascular endothelial function, cardiovascular disease (CVD) risk factors, and physical activity (PA) profiles in people with CF (pwCF) on ETI compared to healthy matched controls. Methods: In 15 pwCF and 15 age- and sex-matched controls, microvascular endothelial function (via transdermal delivery of insulin [INS] and acetylcholine [ACh] on the forearm), macrovascular endothelial function (via flow-mediated dilation [FMD] of the brachial artery), central haemodynamic parameters, including heart rate (HR), stroke volume index (SVi) and cardiac output index (Q̇I) (via thoracic impedance cardiography), body mass index (BMI), blood pressure (BP), and accelerometer-assessed PA, were measured. Results: There were no differences in INS or FMD-mediated vasodilation between the groups (P>0.05). However, a reduced vasodilatory response was evident in pwCF following ACh-mediated vasodilation (P=0.01) and FMD normalised for shear rate (P=0.03). No differences in resting HR, SVi, Q̇I, BP, BMI or PA were found (P>0.05). Conclusion: This study demonstrated reduced micro- and macrovascular function in pwCF. This dysfunction may have potential health implications, particularly regarding long-term cardiovascular risk and further longitudinal assessments are warranted. Journal Article Journal of Cystic Fibrosis 24 4 801 808 Elsevier BV 1569-1993 1873-5010 Cardiovascular; CFTR modulator therapy; Endothelial function; Flow-mediated dilation; Iontophoresis; Physical activity 1 7 2025 2025-07-01 10.1016/j.jcf.2025.02.001 COLLEGE NANME Engineering and Applied Sciences School COLLEGE CODE EAAS Swansea University Another institution paid the OA fee This study was funded by the Cystic Fibrosis Warriors Charity (Charity No 1178063). 2025-09-30T10:56:16.7464937 2025-03-25T13:56:41.1488812 Faculty of Science and Engineering School of Engineering and Applied Sciences - Sport and Exercise Sciences Lauren J Clayton 0000-0002-4907-257x 1 Anthony I Shepherd 0000-0001-6392-7944 2 Jo Corbett 0000-0002-6552-6471 3 Maria Perissiou 0000-0002-3974-2250 4 Gary Connett 5 Julian Legg 6 Mark Allenby 0000-0001-7403-2097 7 Thomas Daniels 0000-0002-5249-5100 8 Don S Urquhart 0000-0002-9048-9692 9 Kelly Mackintosh 0000-0003-0355-6357 10 Melitta McNarry 0000-0003-0813-7477 11 Zoe L Saynor 0000-0003-0674-8477 12 69150__35209__82967f18d2c2490383838d45504c7ba7.pdf 69150.VOR.pdf 2025-09-30T10:49:49.5131133 Output 613220 application/pdf Version of Record true © 2025 The Author(s). This is an open access article distributed under the terms of the Creative Commons CC-BY license. true eng http://creativecommons.org/licenses/by/4.0/
title Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
spellingShingle Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
Kelly Mackintosh
Melitta McNarry
title_short Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
title_full Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
title_fullStr Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
title_full_unstemmed Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
title_sort Cardiovascular function in people with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A cross-sectional, observational, single-centre study
author_id_str_mv bdb20e3f31bcccf95c7bc116070c4214
062f5697ff59f004bc8c713955988398
author_id_fullname_str_mv bdb20e3f31bcccf95c7bc116070c4214_***_Kelly Mackintosh
062f5697ff59f004bc8c713955988398_***_Melitta McNarry
author Kelly Mackintosh
Melitta McNarry
author2 Lauren J Clayton
Anthony I Shepherd
Jo Corbett
Maria Perissiou
Gary Connett
Julian Legg
Mark Allenby
Thomas Daniels
Don S Urquhart
Kelly Mackintosh
Melitta McNarry
Zoe L Saynor
format Journal article
container_title Journal of Cystic Fibrosis
container_volume 24
container_issue 4
container_start_page 801
publishDate 2025
institution Swansea University
issn 1569-1993
1873-5010
doi_str_mv 10.1016/j.jcf.2025.02.001
publisher Elsevier BV
college_str Faculty of Science and Engineering
hierarchytype
hierarchy_top_id facultyofscienceandengineering
hierarchy_top_title Faculty of Science and Engineering
hierarchy_parent_id facultyofscienceandengineering
hierarchy_parent_title Faculty of Science and Engineering
department_str School of Engineering and Applied Sciences - Sport and Exercise Sciences{{{_:::_}}}Faculty of Science and Engineering{{{_:::_}}}School of Engineering and Applied Sciences - Sport and Exercise Sciences
document_store_str 1
active_str 0
description Background: Cystic fibrosis (CF) has been associated with impaired cardiovascular and endothelial function. CF transmembrane conductance regulator (CFTR) modulator therapy, most recently, Elexacaftor/Tezacaftor/Ivacaftor (ETI) has led to improved CFTR function and life expectancy, however, the rising prevalence of obesity in adults is concerning. This study assessed the micro- and macro-vascular endothelial function, cardiovascular disease (CVD) risk factors, and physical activity (PA) profiles in people with CF (pwCF) on ETI compared to healthy matched controls. Methods: In 15 pwCF and 15 age- and sex-matched controls, microvascular endothelial function (via transdermal delivery of insulin [INS] and acetylcholine [ACh] on the forearm), macrovascular endothelial function (via flow-mediated dilation [FMD] of the brachial artery), central haemodynamic parameters, including heart rate (HR), stroke volume index (SVi) and cardiac output index (Q̇I) (via thoracic impedance cardiography), body mass index (BMI), blood pressure (BP), and accelerometer-assessed PA, were measured. Results: There were no differences in INS or FMD-mediated vasodilation between the groups (P>0.05). However, a reduced vasodilatory response was evident in pwCF following ACh-mediated vasodilation (P=0.01) and FMD normalised for shear rate (P=0.03). No differences in resting HR, SVi, Q̇I, BP, BMI or PA were found (P>0.05). Conclusion: This study demonstrated reduced micro- and macrovascular function in pwCF. This dysfunction may have potential health implications, particularly regarding long-term cardiovascular risk and further longitudinal assessments are warranted.
published_date 2025-07-01T05:27:25Z
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