{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T02:40:37Z","timestamp":1790217637026,"version":"4.1.0"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:sec>\n            <jats:title>Background:<\/jats:title>\n            <jats:p>Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods:<\/jats:title>\n            <jats:p>We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia &gt;250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (\u226530s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results:<\/jats:title>\n            <jats:p>\n              A total of 864 patients with definite ARVC (40\u00b116 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77\u201310.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD\/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (\n              <jats:italic>P<\/jats:italic>\n              =0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69\u20130.80) and calibration slope of 0.95 (95% CI, 0.94\u20130.98) indicating minimal over-optimism.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions:<\/jats:title>\n            <jats:p>LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circep.120.008509","type":"journal-article","created":{"date-parts":[[2020,12,9]],"date-time":"2020-12-09T17:01:29Z","timestamp":1607533289000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":113,"title":["Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy"],"prefix":"10.1161","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7489-8744","authenticated-orcid":true,"given":"Julia","family":"Cadrin-Tourigny","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Cardiology (J.C.-T., W.W., A.B., C.T., B.M., S.C., J.E.C., D.P.J., H.T., H.C., C.A.J.), Johns Hopkins Hospital, Baltimore, MD."},{"name":"Cardiovascular Genetics Center, Montreal Heart Institute, Universit\u00e9 de Montr\u00e9al, Canada (J.C.-T., R.T., A.A., M.T., L.R., P.K.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7847-4607","authenticated-orcid":true,"given":"Laurens P.","family":"Bosman","sequence":"additional","affiliation":[{"name":"Netherlands Heart Institute (L.P.B., F.W.A., J.P.v.T., R.N.W.H., A.S.J.M.t.R.)."},{"name":"Department of Cardiology (L.P.B., M.B., F.W.A., J.F.V.d.H., A.S.J.M.t.R.), University Medical Center Utrecht, Utrecht University, the Netherlands."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weijia","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiology (J.C.-T., W.W., A.B., C.T., B.M., S.C., J.E.C., D.P.J., H.T., H.C., C.A.J.), Johns Hopkins Hospital, Baltimore, MD."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1472-0258","authenticated-orcid":true,"given":"Rafik","family":"Tadros","sequence":"additional","affiliation":[{"name":"Cardiovascular Genetics Center, Montreal Heart Institute, Universit\u00e9 de Montr\u00e9al, Canada (J.C.-T., R.T., A.A., M.T., L.R., P.K.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aditya","family":"Bhonsale","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiology (J.C.-T., W.W., A.B., C.T., B.M., S.C., J.E.C., D.P.J., H.T., H.C., C.A.J.), Johns Hopkins Hospital, Baltimore, MD."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mimount","family":"Bourfiss","sequence":"additional","affiliation":[{"name":"Department of Cardiology (L.P.B., M.B., F.W.A., J.F.V.d.H., A.S.J.M.t.R.), University Medical Center Utrecht, Utrecht University, the Netherlands."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d8yvind H.","family":"Lie","sequence":"additional","affiliation":[{"name":"Department of Cardiology and Research group for Cardiogenetics and Sudden Cardiac Death, Oslo University Hospital, Rikshospitalet, Norway (\u00d8.H.L., K.H.H.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1896-0803","authenticated-orcid":true,"given":"Ardan M.","family":"Saguner","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart Center Zurich, Switzerland (A.M.S., F.D.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8404-0541","authenticated-orcid":true,"given":"Anneli","family":"Svensson","sequence":"additional","affiliation":[{"name":"Department of Cardiology and Department of Medical &amp; 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