Cystic fibrosis and the cardiovascular system: the unexpected heartache

In recent years, advancements in medical care and therapies have significantly improved the life expectancy of individuals with cystic fibrosis (CF). As a result, an increasing number of people with CF are now reaching adulthood and experience the long-term consequences of the disease. One of the emerging challenges faced by this growing population is the increased risk of cardiac disease [1]. The rate of cardiovascular disease (CVD) in CF varies among individuals and studies, and the exact prevalence is not well-established. Indeed, a higher body mass index (BMI), lipid metabolism and smoking, all traditional cardiac risk factors, are often attenuated in CF disease [2]. However, it is generally recognised that people with CF have an increased risk of developing cardiovascular complications compared to the general population, which historically has been associated as secondary to progressive lung disease and respiratory failure. Other factors, including presence of CF-related diabetes, high salt dietary intake, chronic kidney disease and chronic inflammation ( i.e. similar to those of other chronic diseases, including HIV, rheumatoid arthritis and systemic lupus erythematosus) all render cardiac risk in this population [2]. Studies have reported varying prevalence rates of CVD, ranging from around 10% to 30% in the CF population. The most common cardiovascular conditions seen in CF patients include atherosclerosis, hypertension and heart failure; however, other clinical sequalae include effects on the aorta, pulmonary hypertension, and peripheral vascular disease [1]. More recently, a multicentre retrospective cohort of people with CF and SARS-CoV-2 infection (n=422) reported that nearly half had a history of diabetes (47%) or hypertension (48%) [3]. Furthermore, 22.5% had a history of ischaemic heart disease, suggesting that CVD might be under-reported in this population. Finally, all reported cardiac risk factors were significantly higher in people with CF compared to those without CF (all p-values <0.01). Consequently, there is a need for proactive cardiac monitoring and management in people with CF to identify and address cardiovascular risk factors early on. Several mechanisms underpin CVD risk in CF [4]. One is via chronic hypoxaemia in CF caused by ventilation heterogeneity and destruction of lung tissue, leading to pulmonary vasoconstriction, pulmonary hypertension and cor pulmonale. A second is through the presence of cystic fibrosis transmembrane conductance regulator (CFTR) protein in cardiac myocytes, including atrial and ventricular myocytes, as well as blood vessels. Similar to its role in epithelial cells, CFTR in the heart plays a role in chloride ion conduction. Finally, the loss of CFTR function can impact myocyte contractility, intracellular calcium signalling, myocardial fibrosis and heart remodelling. Evaluation of the cardiovascular system is important in cystic fibrosis patients

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