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Phenotypic Expression and Genetics of J Wave Syndrome in the Early Stage of Arrhythmogenic Right Ventricular Cardiomyopathy

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J Wave Syndromes

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Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) and J-wave/Brugada syndromes are inherited conditions which predispose to arrhythmic sudden cardiac death. However, they have been traditionally considered separate entities: ARVC is a heart muscle disorder characterized by loss of right ventricular myocardium with fibrofatty substitution while Brugada syndrome is a primary electrical heart disease with no overt structural myocardial abnormalities. Past studies have demonstrated a possible phenotypic overlap suggesting a pathogenetic link between the two diseases. This article reviews the recent scientific evidence coming from cellular and animal experimental studies and genotype-phenotype correlations which may explain the relation between ARVC and J-wave/Brugada syndromes on the basis of a molecular crosstalk between desmosomal-proteins and sodium channel-proteins in the setting of the connexome. Clinical implications of this interrelationship are discussed, with particular reference to the arrhythmic risk during the “concealed phase” of ARVC (i.e., before the structural disease phenotype occurs), when young DS-gene carriers might experience ventricular fibrillation and sudden cardiac death due to a primarily electrical mechanism involving a sodium current reduction, similarly to patients with J-wave/Brugada syndromes.

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Authors and Affiliations

  1. Inherited Arrhythmogenic Cardiomyopathy Unit, Department of Cardiac Thoracic and Vascular Sciences, University of Padova, Via N. Giustiniani 2, 35121, Padova, Italy

    Domenico Corrado MD, PhD

  2. The Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, NY, USA

    Marina Cerrone MD, PhD & Mario Delmar MD, PhD

  3. Department of Cardiac, Thoracic, and Vascular Sciences, University of Padova, Padova, Italy

    Alessandro Zorzi MD

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  1. Domenico Corrado MD, PhD
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  2. Marina Cerrone MD, PhD
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  3. Alessandro Zorzi MD
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  4. Mario Delmar MD, PhD
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Correspondence to Domenico Corrado MD, PhD .

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  1. Cardiovascular Research, Lankenau Institute for Medical Research, Wynnewood, Pennsylvania, USA

    Charles Antzelevitch

  2. Lankenau Institute for Medical Research, Lankenau Medical Center, Wynnewood, Pennsylvania, USA

    Gan-Xin Yan

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Corrado, D., Cerrone, M., Zorzi, A., Delmar, M. (2016). Phenotypic Expression and Genetics of J Wave Syndrome in the Early Stage of Arrhythmogenic Right Ventricular Cardiomyopathy. In: Antzelevitch, C., Yan, GX. (eds) J Wave Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-31578-2_13

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