arrhythmogenic right ventricular dysplasia type 10

(redirected from ARVD10)

arrhythmogenic right ventricular dysplasia type 10

An autosomal dominant disease (OMIM:610193) characterised by partial degeneration of the right ventricular myocardium, electrical instability and sudden death.

Diagnosis
EKG/ECG and angiographic criteria.

Pathology
Fibrofatty replacement of the myocardium of the free wall of the right ventricle.
 
Molecular pathology
Defects in DSG2, which encodes desmoglein 2, a calcium-binding transmembrane glycoprotein component of desmosomes, cause arrhythmogenic right ventricular dysplasia type 10.