{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,11]],"date-time":"2026-07-11T01:22:22Z","timestamp":1783732942629,"version":"3.55.0"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2020,1,13]],"date-time":"2020-01-13T00:00:00Z","timestamp":1578873600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Centro Cardiologico Monzino IRCCS"},{"name":"Registry for Cardio-cerebro-vascular Pathology"},{"name":"Ministry of Health Target Project"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>To provide long-term outcome data on arrhythmogenic cardiomyopathy (ACM) patients with non-classical forms [left dominant ACM (LD-ACM) and biventricular ACM (Bi-ACM)] and an external validation of a recently proposed algorithm for ventricular arrhythmia (VA) prediction in ACM patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Demographic, clinical, and outcome data were retrieved from all ACM patients encountered at our institution. Patients were classified according to disease phenotype (R-ACM; Bi-ACM; LD-ACM). Overall and by phenotype long-term survival were calculated; the novel Cadrin-Tourigny et al. algorithm was used to calculate the a priori predicted VA risk, and it was compared with the observed outcome to test its reliability. One hundred and one patients were enrolled; three subgroups were defined (R-ACM, n\u2009=\u200968; Bi-ACM, n\u2009=\u200914; LD-ACM, n\u2009=\u200919). Over a median of 5.41 (2.59\u20138.37) years, the non-classical form cohort experienced higher rates of VAs than the classical form [5-year freedom from VAs: 0.58 (0.43\u20130.78) vs. 0.76 (0.66\u20130.89), P\u2009=\u20090.04]. The Cadrin-Tourigny et al. predictive model adequately described the overall cohort risk [mean observed-predicted risk difference (O-PRD): +6.7 (\u22124.3, +17.7) %, P\u2009=\u20090.19]; strafing by subgroup, excellent goodness-of-fit was demonstrated for the R-ACM subgroup (mean O-PRD, P\u2009=\u20090.99), while in the Bi-ACM and LD-ACM ones the real observed risk appeared to be underestimated [mean O-PRD: \u221220.0 (\u22121.1, \u221238.9) %, P\u2009&amp;lt;\u20090.0001; \u221222.6 (\u22127.8, \u221237.5) %, P\u2009&amp;lt;\u20090.0001, respectively].<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Non-classical ACM forms appear more prone to VAs than classical forms. The novel prediction model effectively predicted arrhythmic risk in the classical R-ACM cohort, but seemed to underestimate it in non-classical forms.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/europace\/euz352","type":"journal-article","created":{"date-parts":[[2019,12,9]],"date-time":"2019-12-09T12:08:05Z","timestamp":1575893285000},"page":"797-805","source":"Crossref","is-referenced-by-count":41,"title":["Long-term follow-up analysis of a highly characterized arrhythmogenic cardiomyopathy cohort with classical and non-classical phenotypes\u2013a real-world assessment of a novel prediction model: does the subtype really matter"],"prefix":"10.1093","volume":"22","author":[{"given":"Michela","family":"Casella","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3432-070X","authenticated-orcid":false,"given":"Alessio","family":"Gasperetti","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fassini","family":"Gaetano","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattia","family":"Busana","sequence":"first","affiliation":[{"name":"Department of Anesthesiology, Emergency and Intensive Care Medicine, University of G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Sommariva","sequence":"first","affiliation":[{"name":"Unit of Vascular Biology and Regenerative Medicine, Centro Cardiologico Monzino IRCCS, Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Catto","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Sicuso","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Rizzo","sequence":"first","affiliation":[{"name":"Cardiovascular Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Azienda Ospedaliera-University of Padua, Padova (PD), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edoardo","family":"Conte","sequence":"first","affiliation":[{"name":"Dipartimento di Imaging Cardiovascolare, Centro Cardiologico Monzino IRCCS, Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saima","family":"Mushtaq","sequence":"first","affiliation":[{"name":"Dipartimento di Imaging Cardiovascolare, Centro Cardiologico Monzino IRCCS, Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniele","family":"Andreini","sequence":"first","affiliation":[{"name":"Dipartimento di Imaging Cardiovascolare, Centro Cardiologico Monzino IRCCS, Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luigi","family":"Di Biase","sequence":"first","affiliation":[{"name":"Montefiore Medical Center, Albert-Einstein College of Medicine, Bronx, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corrado","family":"Carbucicchio","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Natale","sequence":"first","affiliation":[{"name":"Texas Cardiac Arrhyhtmia Institute (TCAI) at St. David\u2019s Hospital, Austin, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Basso","sequence":"first","affiliation":[{"name":"Cardiovascular Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Azienda Ospedaliera-University of Padua, Padova (PD), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Tondo","sequence":"first","affiliation":[{"name":"Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy"},{"name":"Department of Clinical Sciences and Community Health, University of Milan, Milano (MI), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Dello Russo","sequence":"first","affiliation":[{"name":"Cardiology and Arrhythmology Clinic, Marche Polytechic University, University Hospital \u201cOspedali Riuniti\u201d, Ancona (AN), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,1,13]]},"reference":[{"key":"2020050704575675800_euz352-B1","doi-asserted-by":"crossref","first-page":"2175","DOI":"10.1016\/j.jacc.2008.09.019","article-title":"Left-dominant arrhythmogenic cardiomyopathy","volume":"52","author":"Sen-Chowdhry","year":"2008","journal-title":"J Am Coll Cardiol"},{"key":"2020050704575675800_euz352-B2","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1056\/NEJMra1509267","article-title":"Arrhythmogenic right ventricular cardiomyopathy. 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