arrhythmogenic right ventricular cardiomyopathy type 11; familial arrhythmogenic right ventricular dysplasia type 11 (ARVC11, ARVD11)

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 desmocollin-2 (DSC2)

Clinical manifestations

• ventricular arrhythmias
• heart failure
• sudden death

Diagnostic procedures

• electrocardiogram
• coronary angiography
• clinically defined by electrocardiographic & angiographic criteria

More general terms

• familial arrhythmogenic right ventricular dysplasia

Database

• OMIM: https://mirror.omim.org/entry/610476