arrhythmogenic right ventricular cardiomyopathy type 10; familial arrhythmogenic right ventricular dysplasia type 10 (ARVC10, ARVD10)
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Pathology
- partial degeneration of the myocardium of the right ventricle
- replacement of ventricular myocardium with fatty & fibrous elements
- preferential involvement of the right ventricular free wall
Genetics
- associated with defects in desmoglein-2 (DSG2)
Clinical manifestations
Diagnostic procedures
- electrocardiogram
- coronary angiography
- clinically defined by electrocardiographic & angiographic criteria