Duus Lisa Steen, Dons Maria, Thudium Rebekka Faber, Nielsen Susanne Dam, Olsen Mette F, Qvist Tavs, Lassen Mats C Højbjerg, Skaarup Kristoffer Grundtvig, Johansen Niklas Dyrby, Bluhme Thomas Mørk-Strøm, Katzenstein Terese L, Pressler Tacjana, Faurholt-Jepsen Daniel, Biering-Sørensen Tor
Cardiovascular Non-Invasive Imaging Research Laboratory, Department of Cardiology, Copenhagen University Hospital - Herlev & Gentofte. Hospital, Gentofte Hospitalsvej 8, 2900 Hellerup, University of Copenhagen, Denmark.
Cardiovascular Non-Invasive Imaging Research Laboratory, Department of Cardiology, Copenhagen University Hospital - Herlev & Gentofte. Hospital, Gentofte Hospitalsvej 8, 2900 Hellerup, University of Copenhagen, Denmark; Center for Translational Cardiology and Pragmatic Randomized Trials, Department of Biomedical Sciences, Blegdamsvej 3B, 2200 Copenhagen, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark.
J Cyst Fibros. 2024 Nov;23(6):1138-1145. doi: 10.1016/j.jcf.2024.09.012. Epub 2024 Sep 28.
The extent of cardiac involvement in cystic fibrosis (CF) remains to be determined. The remarkable therapeutic advancements with new highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator treatment and subsequent increase in life expectancy substantiates further research. We aimed to explore the prevalence of cardiac alterations in people with CF (pwCF) compared to matched controls and investigate potential cardiovascular risk factors.
In this cross-sectional study, 104 pwCF underwent clinical and echocardiographic assessment. All participants were matched 1:1 with controls from the general population.
Of 104 pwCF, 44 % were female, mean age was 34 years, and 93 % received CFTR modulator treatment. The prevalence of abnormal cardiac function in pwCF was 44 %, more than double the prevalence in controls. PwCF were found to have smaller left ventricular (LV) dimensions, worse LV diastolic function, and reduced right ventricle (RV) as well as LV systolic function. After multivariable adjustment, LV diastolic function as well as LV and RV systolic function remained poorer in pwCF as compared to controls. Male sex and decreasing FEV1/FVC ratio remained independently associated with abnormal cardiac function in pwCF (male sex: OR 3.94 (1.56; 9.95), p = 0.004 and FEV1/FVC ratio: OR 2.05 per 0.1 unit decrease (1.21; 3.52), p = 0.008, respectively).
Both left- and right-sided cardiac alterations were found in pwCF. After adjustments for risk factors, both RV and LV systolic measures remained altered in pwCF, compared to controls. Male sex and decreasing pulmonary function evaluated by FEV1/FVC-ratio were associated with abnormal cardiac function in pwCF.
