arrhythmogenic cardiomyopathy
Also written as: ARVC — arrhythmogenic cardiomyopathy
Inherited heart muscle disease characterized by replacement of myocardium with fibrous and fatty tissue, leading to arrhythmias.
Full Definition
A genetic cardiomyopathy primarily affecting the right ventricle, though biventricular involvement is increasingly recognized. The condition is characterized by progressive loss of myocytes with replacement by fibrofatty tissue, particularly in the subepicardial regions. This structural abnormality creates a substrate for ventricular arrhythmias and sudden cardiac death, especially in young athletes. Pathological diagnosis requires specific histological criteria including myocyte loss and fibrofatty replacement exceeding normal limits.
Usage
Usage note: Preferred term over 'arrhythmogenic right ventricular cardiomyopathy' due to recognition of biventricular involvement.
In Context
- "Endomyocardial biopsy confirmed arrhythmogenic cardiomyopathy with extensive fibrofatty infiltration." — Specialized pathology report
- "Family screening identified a pathogenic variant associated with arrhythmogenic cardiomyopathy." — Genetic testing report