arrhythmogenic right ventricular dysplasia—familial—10

Home » Classic Medicine » Syndromes » arrhythmogenic right ventricular dysplasia—familial—10
arrhythmogenic right ventricular dysplasia—familial—102016-11-24T20:34:03+00:00

arrhythmogenic right ventricular dysplasia—familial—10 


An autosomal dominant condition (OMIM:610193) characterized by fibrofatty infiltration and degeneration of the right ventricular myocardium, resulting in ventricular and supraventricular arrhythmias, and sudden death.

Diagnosis EKG/ECG, angiographic criteria

Pathology Fibrofatty replacement of the right ventricle’s free wall myocardium.

Molecular pathology Defects in DSG2, which encodes desmoglein 2, a calcium-binding transmembrane glycoprotein component of desmosomes, cause arrhythmogenic right ventricular dysplasia—familial—10.  

Synonyms Arrhythmogenic right ventricular cardiomyopathy 10, ARVC10, ARVD10 


Leave A Comment

This site uses Akismet to reduce spam. Learn how your comment data is processed.