{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:19:32Z","timestamp":1787008772880,"version":"build-2736575974"},"reference-count":35,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>This study aimed to evaluate phenotypic progression in a large cohort of patients with arrhythmogenic cardiomyopathy (ACM) using cardiac magnetic resonance (CMR) to assess the prognostic impact of baseline structural abnormalities and to investigate the impact of exercise performed after diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>We retrospectively analysed individuals with a definitive diagnosis of ACM or identified as carriers of pathogenic or likely pathogenic (P\/LP) ACM-related genetic variants, all of whom underwent at least one CMR. Longitudinal data on structural remodelling were available for a subset of patients with at least two CMRs. Patients were stratified into higher- and lower-exercise groups based on exercise performed after diagnosis. The primary composite endpoint included major ventricular arrhythmias (MVA), appropriate implantable cardioverter-defibrillator interventions, sudden cardiac arrest, and sudden cardiac death. A total of 202 patients (mean age: 41.7 \u00b1 16.4 years, 59% males) were included. Over a mean follow-up of 5.2 years, 32 patients (16%) reached the primary endpoint. Baseline myocardial fibrosis (MF) and reduced right ventricular ejection fraction independently predicted arrhythmic events [HR 4.9 (95% CI: 1.8\u201312.9), P &amp;lt; 0.001, and HR 2.6 (95% CI: 1.2\u20135.4), P = 0.002, respectively]. Among the 97 patients with at least two CMR scans, 32% developed de novo fibrosis, and 41% showed progression of MF. Patients with longer intervals between CMR scans demonstrated more pronounced structural remodelling, evidenced by greater increases in biventricular end-diastolic volumes and progressive decline in ventricular function. Significant left ventricular dilation was observed in the higher-exercise group, with an increase in indexed end-diastolic volumes. Carriers of PKP2 variants engaging in moderate-to-intense exercise exhibited a higher incidence of arrhythmic events.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>MF and right ventricular systolic dysfunction emerge as independent predictors of arrhythmic risk in ACM. Serial CMR assessments demonstrate that structural progression is common, supporting the concept that ACM represents a dynamic rather than a static disease process. Patients carrying PKP2 variants may exhibit heightened susceptibility to exercise-related arrhythmic risk.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/eurjpc\/zwag130","type":"journal-article","created":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T12:47:07Z","timestamp":1773233227000},"source":"Crossref","is-referenced-by-count":2,"title":["Disease progression in arrhythmogenic cardiomyopathy: a cardiac magnetic resonance longitudinal study"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-9910-387X","authenticated-orcid":false,"given":"Tiziana Cristina","family":"Minopoli","sequence":"first","affiliation":[{"name":"Cardiovascular Clinical Academic Group, City St George\u2019s, University of London, and St George\u2019s University Hospitals NHS Foundation Trust , Blackshaw Road, Tooting, London SW17 0QT ,","place":["UK"]},{"name":"Department of Experimental and Clinical Medicine, University of Florence , Florence 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