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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. 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. 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]

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.

BACKGROUND: There are few examples of interventions designed to promote physical activity (PA) in adults with Cystic fibrosis (CF). Increasing levels of habitual PA may be more feasible and result in greater compliance than conventional exercise training inventions which give little or no attention to long-term PA behaviour. Despite this there is limited research exploring perceptions of PA among adults with CF. The study aimed to understand the ecological correlates of PA in adults with CF and to involve individuals with CF, their families (where applicable) and clinicians in a formative process to inform the development of an ecological approach to PA promotion in this population. METHODS: An iterative approach was utilised, whereby findings from earlier phases of the research informed subsequent phases. Semi-structured interviews were conducted to explore patients' perceptions of PA, devised using the PRECEDE component of the PRECEDE-PROCEED model. Followed by, focus groups to discuss the perceived barriers, facilitators and opportunities for PA participation and how this information could inform the development and delivery of a PA intervention. Separate focus groups were conducted with individuals with CF (n = 11) and their families and CF MDT members. Thematic analysis was used to construct themes. RESULTS: Physical and mental wellbeing manifested as both barriers and facilitators of PA. CF is characterised by a progressive decline in physical function, which presents as a number of challenging symptoms and set-backs for an individual with CF. PA represents an opportunity for participants to slow the rate of this decline and manage the symptoms associated with the condition. Enjoyment was an important facilitator of PA. Exercise professionals and family reinforce PA behaviour, particularly during adolescence. CONCLUSIONS: PA promotion should form part of routine CF care with additional exercise professional support during adolescence.

Freddy Frost,1 Paula Dyce,2 Alicja Ochota,3 Sejal Pandya,3 Thomas Clarke,3 Martin J Walshaw,1 Dilip S Nazareth11Respiratory Medicine, Adult CF Centre, Liverpool Heart & Chest Hospital, Liverpool L14 3PE, UK; 2Cystic Fibrosis Related Diabetes Service, Adult CF Centre, Liverpool Heart & Chest Hospital, Liverpool L14 3PE, UK; 3Adult CF Centre, Liverpool Heart & Chest Hospital, Liverpool, L14 3PE, UKAbstract: Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis and can be present in over 50% of adults with the disease. CFRD is associated with poorer clinical outcomes, including accelerated pulmonary function decline and excess morbidity. The management of CFRD is complex and differs from that of type 1 and type 2 diabetes mellitus such that clinicians responsible for the care of people with CFRD must work closely with colleagues across a number of different specialities and disciplines. This review aims to discuss why a multi-disciplinary approach is important and how it can be harnessed to optimize the care of people with CFRD.Keywords: insulin, cystic fibrosis, hyperglycemia, nursing, diagnostics, teamwork