Education in Heart
CARDIOMYOPATHYArrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment
Domenico Corrado, Cristina Basso, Gaetano Thiene
Departments of Cardiology and Pathology, University of Padova,
Italy
Correspondence to: Domenico Corrado MD, Department of Cardiology, University of Padua Medical School, Via Giustiniani 2 - 35121 Padova, Italy email: cardpath@ux1.unipd.it
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Introduction |
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Arrhythmogenic
right ventricular cardiomyopathy (ARVC) is a myocardial disease, often
familial, that is characterised pathologically by fibrofatty
replacement of the right ventricular myocardium, and clinically by
ventricular arrhythmias of right ventricular origin which may lead to
sudden death, mostly in young people and athletes.1-5 The
term "dysplasia" was originally used to describe an entity that was
considered to be the result of a developmental defect of the right
ventricular myocardium.1 A better understanding of
clinical manifestations as well as morphologic findings does not
support the theory of a congenital absence of the myocardium, but is in
keeping with a non-ischaemic, ongoing atrophy of the right ventricular
myocardium, most likely genetically determined, which becomes
symptomatic in adolescents and young adults.2-4 On the
basis of its nature of progressive heart muscle disease of unknown
aetiology, ARVC has been more appropriately included among the
cardiomyopathies in the recent classification proposed by the task
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