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Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a progressive cardiomyopathy with different clinical-pathological patterns: (a) “silent” cardiomyopathic abnormalities localized in the right ventricle in asymptomatic victims of sudden death; (b) “overt” disease characterized by segmental or global right ventricular structural changes, often associated with histological evidence of left ventricular involvement and underlying symptomatic ventricular arrhythmias; and (c) “end-stage” biventricular cardiomyopathy mimicking dilated cardiomyopathy, leading to progressive heart failure and eventually requiring heart transplantation [1]. A scoring system to establish the diagnosis of ARVC/D has been developed on the basis of the presence of major and minor criteria encompassing structural, histological, electrocardiographic, arrhythmic, and genetic features of the disease [2].

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Rampazzo, A., Danieli, G.A. (2007). Advances in Genetics: Dominant Forms. In: Markus, F.I., Nava, A., Thiene, G. (eds) Arrhythmogenic RV Cardiomyopathy/Dysplasia. Springer, Milano. https://doi.org/10.1007/978-88-470-0490-0_2

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  • DOI: https://doi.org/10.1007/978-88-470-0490-0_2

  • Publisher Name: Springer, Milano

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