Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
ARVD8
Introduction
Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 8; arrhythmogenic right ventricular cardiomyopathy 8; ARVC8
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 (607450) is associated with a mutation in the DSP gene (125647) on chromosome 6p24.[1]
Epidemiology and Demographics
Epidemiology and Demographics
Natural History, Complications, Prognosis
Natural History, Complications, Prognosis
Diagnosis
Diagnosis
Symptoms
Electrocardiogram
Echocardiogram
MRI
References
References
- ↑ Rampazzo A, Nava A, Malacrida S et-al. Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am. J. Hum. Genet. 2002;71 (5): 1200-6. doi:10.1086/344208 – Free text at pubmed – Pubmed citation
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]