{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T20:35:28Z","timestamp":1792096528122,"version":"4.4.0"},"reference-count":37,"publisher":"European Respiratory Society (ERS)","issue":"4","license":[{"start":{"date-parts":[[2023,7,20]],"date-time":"2023-07-20T00:00:00Z","timestamp":1689811200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2023,7,5]]},"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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 CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We looked at rates and outcomes for cardiac disease in 5649 adult PwCF in the UK CF Registry and 6265 adult PwCF in TriNetX (a global federated database of electronic healthcare record data). We used propensity matching to compare risk of major adverse cardiac events (MACE) (myocardial infarction, left-sided heart failure and atrial fibrillation) in PwCF against matched non-CF comparators in the general population and other inflammatory diseases.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>PwCF had a high prevalence of diabetes but low rates of hypertension and obesity. Some cardiac risk factors (age, diabetes and hypertension) were associated with MACE, but relationships between disease-specific risk factors (lung function and intravenous antibiotic days) were also observed. In propensity score-matched analyses, PwCF had higher risk of MACE than matched general population comparators (hazard ratio (HR) 1.65, 95% CI 1.40\u20131.95; p&lt;0.001) and an equivalent or higher relative risk compared with other inflammatory conditions considered \u201chigh risk\u201d for cardiovascular disease, including systemic lupus erythematosus (HR 0.95, 95% CI 0.82\u20131.09; p=0.44), rheumatoid arthritis (HR 1.21, 95% CI 1.00\u20131.48; p&lt;0.001) and HIV (HR 0.93, 95% CI 0.82\u20131.06; p=0.29).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1183\/13993003.00174-2023","type":"journal-article","created":{"date-parts":[[2023,7,20]],"date-time":"2023-07-20T11:32:50Z","timestamp":1689852770000},"page":"2300174","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":48,"title":["Prevalence, risk factors and outcomes of cardiac disease in cystic fibrosis: a multinational retrospective cohort study"],"prefix":"10.1183","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3902-6502","authenticated-orcid":false,"given":"Freddy","family":"Frost","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6114-0467","authenticated-orcid":false,"given":"Dilip","family":"Nazareth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9267-1658","authenticated-orcid":false,"given":"Laurent","family":"Fauchier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis","family":"Wat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Shelley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Austin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin J.","family":"Walshaw","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory Y.H.","family":"Lip","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2023,7,20]]},"reference":[{"key":"2024102103165442000_62.4.2300174.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"2024102103165442000_62.4.2300174.2","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2020-216265"},{"key":"2024102103165442000_62.4.2300174.3","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/j.jcf.2021.11.008","article-title":"Forewarned is forearmed: the cardiovascular time bomb in cystic fibrosis","volume":"21","author":"Duckers","year":"2022","journal-title":"J Cyst Fibros"},{"key":"2024102103165442000_62.4.2300174.4","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1038\/s44161-022-00030-y","article-title":"Identifying and preventing cardiovascular disease in patients with cystic fibrosis","volume":"1","author":"Saunders","year":"2022","journal-title":"Nat Cardiovasc Res"},{"key":"2024102103165442000_62.4.2300174.5","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.jcf.2021.04.016","article-title":"Cardiovascular complications in cystic fibrosis: a review of the literature","volume":"21","author":"Poore","year":"2022","journal-title":"J Cyst Fibros"},{"key":"2024102103165442000_62.4.2300174.6","doi-asserted-by":"publisher","DOI":"10.2174\/157340308784245775"},{"key":"2024102103165442000_62.4.2300174.7","doi-asserted-by":"crossref","first-page":"e58","DOI":"10.1016\/S2665-9913(20)30221-6","article-title":"Cardiovascular risk in inflammatory arthritis: rheumatoid arthritis and gout","volume":"3","author":"Hansildaar","year":"2021","journal-title":"Lancet Rheumatol"},{"key":"2024102103165442000_62.4.2300174.8","doi-asserted-by":"crossref","first-page":"e279","DOI":"10.1016\/S2352-3018(20)30036-9","article-title":"HIV and cardiovascular disease","volume":"7","author":"So-Armah","year":"2020","journal-title":"Lancet HIV"},{"key":"2024102103165442000_62.4.2300174.9","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/j.jcf.2021.11.012","article-title":"Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis","volume":"21","author":"Petersen","year":"2022","journal-title":"J Cyst Fibros"},{"key":"2024102103165442000_62.4.2300174.10","doi-asserted-by":"crossref","first-page":"106727","DOI":"10.1016\/j.rmed.2021.106727","article-title":"When is cystic fibrosis not cystic fibrosis? 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