INTRODUCTION: Although people living with cystic fibrosis (PwCF) often have some risk factors for cardiovascular disease including diabetes and chronic inflammation, little is known about the long-term cardiac risk in this condition. We aimed to determine the characteristics, rates, and outcomes for cardiac disease in cystic fibrosis.

METHODS: We looked at rates and outcomes for cardiac disease in 5649 adult PwCF in the UK CF Registry and 6265 in TriNetX (a global federated database of electronic healthcare record data). We used propensity-matching to compare risk of major adverse cardiac events (myocardial infarction, left-sided heart failure, atrial fibrillation; MACE) in PwCF against matched non-CF comparators in the general population and other inflammatory diseases.

RESULTS: PwCF had high prevalence of diabetes but low rates of hypertension and obesity. Some cardiac risk factors (age, diabetes, hypertension) were associated with MACE, but relationships between disease specific risk factors (lung function and intravenous antibiotic days) were also observed.In propensity-matched analyses, PwCF had higher risk of MACE than matched general population comparators (Hazard Ratio [95% CI] 1.65 [1.40 to 1.95], p<0.001), and an equivalent or higher relative risk compared to other inflammatory conditions considered "high-risk" for cardiovascular disease including rheumatoid arthritis (HR 1.21 [1.00 to 1.48], p<0.001), systemic lupus erythematosus (RR 0.95 [0.82 to 1.09], p=0.44) and human immunodeficiency virus (HR 0.93 [0.82 to 1.06], p=0.29) CONCLUSION: PwCF are at increased risk of adverse cardiac disease events. Future work should focus on defining determinants of cardiovascular risk such that appropriate risk stratification can be employed.