familial arrhythmogenic right ventricular dysplasia 8 (ARVD8)
Jump to navigation
Jump to search
Pathology
- replacement of ventricular myocardium with fatty & fibrous elements
- partial degeneration of the myocardium of the right ventricle
- preferential involvement of the right ventricular free wall
Genetics
- autosomal dominant
- associated with defects in DSP
Clinical manifestations
- electrical instability
- sudden death
Diagnostic criteria
- clinically defined by:
- electrocardiographic & angiographic criteria
- pathologic findings