ARVD4

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

Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 4; arrhythmogenic right ventricular cardiomyopathy 4; ARVC4

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. This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and left bundle branch block.

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. This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and left bundle branch block.

Genetics

This variant (602087) is associated with a mutation in the chromosome 2q32.1-q32.3 region.^[1]

Epidemiology and Demographics

Natural History, Complications, Prognosis

Diagnosis

Symptoms

Electrocardiogram

This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and left bundle branch block.

Echocardiogram

MRI

References

↑ Rampazzo A, Nava A, Miorin M et-al. ARVD4, a new locus for arrhythmogenic right ventricular cardiomyopathy, maps to chromosome 2 long arm. Genomics. 1997;45 (2): 259-63. doi:10.1006/geno.1997.4927 – Pubmed citation

CME Category::Cardiology

[1] Rampazzo A, Nava A, Miorin M et-al. ARVD4, a new locus for arrhythmogenic right ventricular cardiomyopathy, maps to chromosome 2 long arm. Genomics. 1997;45 (2): 259-63. doi:10.1006/geno.1997.4927 – Pubmed citation

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