ARVC
Also written as: ARVC — arrhythmogenic right ventricular cardiomyopathy
Inherited cardiomyopathy characterized by fibrofatty replacement of right ventricular myocardium and associated with arrhythmias and sudden death.
Full Definition
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease characterized by progressive fibrofatty replacement of the right ventricular myocardium, though left ventricular involvement may also occur. The condition is caused by mutations in genes encoding desmosomal proteins, leading to defective cell-to-cell adhesion and subsequent myocyte loss with replacement by fibrous and adipose tissue. Pathologically, the disease shows a characteristic triangle of dysplasia affecting the right ventricular inflow tract, outflow tract, and apex. The fibrofatty infiltration predisposes to ventricular arrhythmias and sudden cardiac death, particularly in young athletes. Diagnosis requires integration of clinical, electrocardiographic, imaging, and sometimes pathologic criteria.
Usage
Usage note: ARVC is now preferred over ARVD; always specify if left ventricular involvement is present.
In Context
- "Autopsy findings were consistent with ARVC, showing extensive fibrofatty replacement of the right ventricular free wall." — Sudden death autopsy
- "The endomyocardial biopsy confirmed the clinical suspicion of ARVC with characteristic fibrofatty changes." — Cardiac pathology report