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Minerva Cardioangiologica 2014 October;62(5):369-78


lingua: Inglese

Anatomical features and clinical correlations in Caucasian patients with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy

Inciardi R. M. 1, Maresi E. 2, Coppola G. 1, Rotolo A. 1, Clemenza F. 3, Giordano U. 4, Lombardo E. 5, Schicchi R. 6, Torcivia R. 7, Arrotti S. 1, Iacona R. 1, Minacapelli A. A. 1, Assennato P. 1, Novo S. 1

1 UOC Cardiologia II con Emodinamica, “P. Giaccone” Hospital, University of Palermo, Palermo, Italy; 2 Department of Legal Medicine, “P. Giaccone” Hospital, University of Palermo, Palermo, Italy; 3 Heart Failure Unit, ISMETT, Palermo, Italy; 4 Department of Cardiology, ARNAS Ospedale Civico, Palermo, Italy; 5 Department of Cardiology, Maria Eleonora Hospital, Palermo, Italy; 6 Division of Cardiology, Buccheri La Ferla Fatebenefratelli Hospital, Palermo, Italy; 7 Cardiology Unit, Fondazione Istituto S. Raffaele‑G. Giglio, Cefalù, Italy


AIM: Arrhythmogenic right ventrticular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by fibrofatty replacement and a high risk of ventricular arrhythmias (VA) and sudden cardiac death (SCD). The aim of the present investigation is to examine the pathological profile and the clinical correlations in a group of ARVD/C patients.
METHODS: We conducted a multicenter study evaluating 47 patients (31 men; mean age 37±14 years) with definite ARVD/C. Diagnosis was established according to the actual clinicomorphologic criteria at autopsy or clinically. We divided the study population in 2 different groups. First group included 28 alive patients and the second 19 patients dead suddenly.
RESULTS: Age at presentation was different in the two groups (P=0.0015). We observed an important association regarding the risk of sudden death and the history of physical exercise (P=0.0017). Moreover patients with negative outcome (i.e., SCD, cardiac transplantation, congestive heart failure) had a significantly association with biventricular form of ARVD/C (P=0.0034) and age presentation (P=0.003). Left ventricular (LV) involvement was frequently observed in the two groups (17% and 32% respectively). Post-mortem examination revealed frequent inflammatory infiltrates (26%) indicating active myocarditis, which probably justify the fatal arrhythmic events occurred in these patients.
CONCLUSION: Frequent LV involvement justifies the recent adoption of the broad term Arrhythmogenic Cardiomyopathy. Early age presentation, sport activity and the biventricular form of ARVD/C represent important predictors of adverse outcome that can be useful to early identify patients at high risk.

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