ARVD9
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 9; arrhythmogenic right ventricular cardiomyopathy 9; ARVC9
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 (609040) is associated with a mutation in the PKP2 gene (602861) on chromosome 12p11.[1]
Epidemiology and Demographics
Epidemiology and Demographics
Natural History, Complications, Prognosis
Natural History, Complications, Prognosis
Diagnosis
Diagnosis
Symptoms
Electrocardiogram
Echocardiogram
MRI
References
References
- ↑ Grossmann KS, Grund C, Huelsken J et-al. Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation. J. Cell Biol. 2004;167 (1): 149-60. doi:10.1083/jcb.200402096 – Free text at pubmed – Pubmed citation
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