Naxos syndrome is associated with which form of cardiomyopathy?

Naxos syndrome is associated with which form of cardiomyopathy?

• A. Hypertrophic cardiomyopathy
• B. Dilated cardiomyopathy (DCM)
• C. Restrictive cardiomyopathy
• D. Arrhythmogenic right ventricular cardiomyopathy

Answer: (B)

Naxos syndrome is associated with arrhythmogenic right ventricular cardiomyopathy. It’s a syndromic form of ARVC characterized by fibrofatty replacement of the right ventricular myocardium, often with distinctive skin and hair findings (woolly hair and palmoplantar keratoderma). Mutations in desmosomal proteins disrupt cell–cell adhesion in cardiomyocytes, promoting RV dilation, arrhythmias, and risk of sudden death. This contrasts with dilated cardiomyopathy, which involves dilation and systolic dysfunction of the ventricles (often LV-dominant). So the correct association is arrhythmogenic right ventricular cardiomyopathy.

Naxos syndrome is associated with arrhythmogenic right ventricular cardiomyopathy. It’s a syndromic form of ARVC characterized by fibrofatty replacement of the right ventricular myocardium, often with distinctive skin and hair findings (woolly hair and palmoplantar keratoderma). Mutations in desmosomal proteins disrupt cell–cell adhesion in cardiomyocytes, promoting RV dilation, arrhythmias, and risk of sudden death. This contrasts with dilated cardiomyopathy, which involves dilation and systolic dysfunction of the ventricles (often LV-dominant). So the correct association is arrhythmogenic right ventricular cardiomyopathy.