{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T16:03:09Z","timestamp":1787241789544,"version":"build-2736575974"},"reference-count":24,"publisher":"European Respiratory Society (ERS)","issue":"5","license":[{"start":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T00:00:00Z","timestamp":1692230400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100009708","name":"Novo Nordisk Fonden","doi-asserted-by":"publisher","award":["NNF19OC0058811"],"award-info":[{"award-number":["NNF19OC0058811"]}],"id":[{"id":"10.13039\/501100009708","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001448","name":"Alpha-1 Foundation","doi-asserted-by":"publisher","award":["612336"],"award-info":[{"award-number":["612336"]}],"id":[{"id":"10.13039\/100001448","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009457","name":"Region Sj\u00e6lland","doi-asserted-by":"publisher","award":["R19A341B233"],"award-info":[{"award-number":["R19A341B233"]}],"id":[{"id":"10.13039\/501100009457","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2023,7,31]]},"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Individuals with \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency have increased elastase activity resulting in continuous degradation of elastin and early onset of COPD. Increased elastase activity may also affect elastic properties of the heart, which may impact risk of heart failure. We tested the hypothesis that \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency is associated with increased risk of heart failure in two large populations.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In a nationwide nested study of 2209 patients with \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency and 21\u2009869 controls without \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency matched on age, sex and municipality, we recorded admissions and deaths due to heart failure during a median follow-up of 62\u2005years. We also studied a population-based cohort of another 102\u2009481 individuals from the Copenhagen General Population Study including 187 patients from the Danish \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -Antitrypsin Deficiency Registry, all with genetically confirmed \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Individuals with\n                      <jats:italic>versus<\/jats:italic>\n                      without \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency had increased risk of heart failure hospitalisation in the nationwide cohort (adjusted hazard ratio 2.64, 95% CI 2.25\u20133.10) and in the population-based cohort (1.77, 95% CI 1.14\u20132.74). Nationwide, these hazard ratios were highest in those without myocardial infarction (3.24, 95% CI 2.70\u20133.90), without aortic valve stenosis (2.80, 95% CI 2.38\u20133.29), without hypertension (3.44, 95% CI 2.81\u20134.22), without atrial fibrillation (3.33, 95% CI 2.75\u20134.04) and without any of these four diseases (6.00, 95% CI 4.60\u20137.82). Hazard ratios for heart failure-specific mortality in individuals with\n                      <jats:italic>versus<\/jats:italic>\n                      without \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency were 2.28 (95% CI 1.57\u20133.32) in the nationwide cohort and 3.35 (95% CI 1.04\u201310.74) in the population-based cohort.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Individuals with \u03b1\n                      <jats:sub>1<\/jats:sub>\n                      -antitrypsin deficiency have increased risk of heart failure hospitalisation and heart failure-specific mortality in the Danish population.\n                    <\/jats:p>\n                  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