eprintid: 10082335 rev_number: 18 eprint_status: archive userid: 608 dir: disk0/10/08/23/35 datestamp: 2019-09-26 09:07:54 lastmod: 2021-11-30 23:03:28 status_changed: 2019-09-26 09:07:54 type: article metadata_visibility: show creators_name: Cadrin-Tourigny, J creators_name: Bosman, LP creators_name: Nozza, A creators_name: Wang, W creators_name: Tadros, R creators_name: Bhonsale, A creators_name: Bourfiss, M creators_name: Fortier, A creators_name: Lie, ØH creators_name: Saguner, AM creators_name: Svensson, A creators_name: Andorin, A creators_name: Tichnell, C creators_name: Murray, B creators_name: Zeppenfeld, K creators_name: van den Berg, MP creators_name: Asselbergs, FW creators_name: Wilde, AAM creators_name: Krahn, AD creators_name: Talajic, M creators_name: Rivard, L creators_name: Chelko, S creators_name: Zimmerman, SL creators_name: Kamel, IR creators_name: Crosson, JE creators_name: Judge, DP creators_name: Yap, S-C creators_name: van der Heijden, JF creators_name: Tandri, H creators_name: Jongbloed, JDH creators_name: Guertin, M-C creators_name: van Tintelen, JP creators_name: Platonov, PG creators_name: Duru, F creators_name: Haugaa, KH creators_name: Khairy, P creators_name: Hauer, RNW creators_name: Calkins, H creators_name: Te Riele, ASJM creators_name: James, CA title: A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy ispublished: pub divisions: UCL divisions: B02 divisions: DD4 keywords: Arrhythmogenic right ventricular cardiomyopathy, Implantable cardioverter-defibrillators, Sudden cardiac death, Ventricular arrhythmias note: This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited abstract: Aims Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. Methods and results Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44–9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73–0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92–0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P < 0.001). Conclusion Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs date: 2019-06-14 date_type: published official_url: https://doi.org/10.1093/eurheartj/ehz103 oa_status: green full_text_type: pub pmcid: PMC6568197 language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 1673895 doi: 10.1093/eurheartj/ehz103 pii: 5419784 lyricists_name: Asselbergs, Folkert lyricists_id: FASSE08 actors_name: Kalinowski, Damian actors_id: DKALI47 actors_role: owner full_text_status: public publication: European Heart Journal volume: 40 number: 23 pagerange: 1850-1858 event_location: England issn: 1522-9645 citation: Cadrin-Tourigny, J; Bosman, LP; Nozza, A; Wang, W; Tadros, R; Bhonsale, A; Bourfiss, M; ... James, CA; + view all <#> Cadrin-Tourigny, J; Bosman, LP; Nozza, A; Wang, W; Tadros, R; Bhonsale, A; Bourfiss, M; Fortier, A; Lie, ØH; Saguner, AM; Svensson, A; Andorin, A; Tichnell, C; Murray, B; Zeppenfeld, K; van den Berg, MP; Asselbergs, FW; Wilde, AAM; Krahn, AD; Talajic, M; Rivard, L; Chelko, S; Zimmerman, SL; Kamel, IR; Crosson, JE; Judge, DP; Yap, S-C; van der Heijden, JF; Tandri, H; Jongbloed, JDH; Guertin, M-C; van Tintelen, JP; Platonov, PG; Duru, F; Haugaa, KH; Khairy, P; Hauer, RNW; Calkins, H; Te Riele, ASJM; James, CA; - view fewer <#> (2019) A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. European Heart Journal , 40 (23) pp. 1850-1858. 10.1093/eurheartj/ehz103 <https://doi.org/10.1093/eurheartj%2Fehz103>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10082335/1/Asselbergs_A%20new%20prediction%20model%20for%20ventricular%20arrhythmias%20in%20arrhythmogenic%20right%20ventricular%20cardiomyopathy_VoR.pdf