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ARVD6

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

Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 6; arrhythmogenic right ventricular cardiomyopathy 6; ARVC6

Overview

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

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 (604401), is associated with a mutation in the chromosome 10p14-p12 region.[1]

Epidemiology and Demographics

Epidemiology and Demographics

Natural History, Complications, Prognosis

Natural History, Complications, Prognosis

Diagnosis

Diagnosis

Symptoms

Electrocardiogram

Echocardiogram

MRI

References

References

  1. Li D, Ahmad F, Gardner MJ et-al. The locus of a novel gene responsible for arrhythmogenic right-ventricular dysplasia characterized by early onset and high penetrance maps to chromosome 10p12-p14. Am. J. Hum. Genet. 2000;66 (1): 148-56. doi:10.1086/302713Free text at pubmedPubmed citation

CME Category::Cardiology

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