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familial arrhythmogenic right ventricular dysplasia 8 (ARVD8)
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
Diagnosis:
- clinically defined by:
a) electrocardiographic & angiographic criteria
b) pathologic findings
Special laboratory:
- electrocardiogram
- coronary angiography
General
familial arrhythmogenic right ventricular dysplasia; arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD, ARVC, ARVD/C, ARVC/D)
Database Correlations
OMIM 607450
References
OMIM :accession 607450