Which syndrome is associated with DCM?

Which syndrome is associated with DCM?

• A. Brugada syndrome
• B. Marfan syndrome
• C. Alport syndrome
• D. Naxos syndrome

Answer: (D)

Dilated cardiomyopathy can be seen as part of certain inherited syndromes that include a cardiomyopathy component along with other distinctive features. Naxos syndrome is one such condition, known for its combination of cardiomyopathy with skin and hair findings—specifically, woolly hair and palmoplantar keratoderma. It results from mutations in desmosomal proteins, which disrupt cell–cell adhesion in the heart and skin, leading to a cardiomyopathic process that can present with ventricular dilation and dysfunction. This makes it the best fit among the options for a syndrome associated with DCM. Brugada syndrome is a channelopathy with risk of sudden death but no structural heart disease component. Marfan syndrome primarily involves connective tissue defects with alect aortic root dilation and other systemic features, not a classic DCM association. Alport syndrome is a kidney and hearing disorder from basement membrane defects, not a cardiomyopathy.

Dilated cardiomyopathy can be seen as part of certain inherited syndromes that include a cardiomyopathy component along with other distinctive features. Naxos syndrome is one such condition, known for its combination of cardiomyopathy with skin and hair findings—specifically, woolly hair and palmoplantar keratoderma. It results from mutations in desmosomal proteins, which disrupt cell–cell adhesion in the heart and skin, leading to a cardiomyopathic process that can present with ventricular dilation and dysfunction. This makes it the best fit among the options for a syndrome associated with DCM.

Brugada syndrome is a channelopathy with risk of sudden death but no structural heart disease component. Marfan syndrome primarily involves connective tissue defects with alect aortic root dilation and other systemic features, not a classic DCM association. Alport syndrome is a kidney and hearing disorder from basement membrane defects, not a cardiomyopathy.