ARVD7

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Arrhythmogenic right ventricular dysplasia Microchapters

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Overview

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Pathophysiology

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Differentiating Arrhythmogenic right ventricular dysplasia from other Diseases

Epidemiology and Demographics

Risk Factors

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Natural History, Complications and Prognosis

Diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 7; arrhythmogenic right ventricular cardiomyopathy 7; ARVC7

Overview

Arrhythmogenic right ventricular dysplasia is a type of nonischemic cardiomyopathy that involves primarily the right ventricle. It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.

Pathophysiology

The pathogenesis of ARVD involves apoptosis with fatty and fibro-fatty infiltration of the right ventricular free wall leading to heart failure and ventricular arrhythmias.

Genetics

This variant (609160) is associated with a mutation in the chromosome 10q22.3 region.[1]

Epidemiology and Demographics

Natural History, Complications, Prognosis

Diagnosis

Symptoms

Electrocardiogram

Echocardiogram

MRI

References

  1. Kuhl A, Melberg A, Meinl E et-al. Myofibrillar myopathy with arrhythmogenic right ventricular cardiomyopathy 7: corroboration and narrowing of the critical region on 10q22.3. Eur. J. Hum. Genet. 2008;16 (3): 367-73. doi:10.1038/sj.ejhg.5201980 - Pubmed citation

CME Category::Cardiology