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Neurocutaneous Disorders

 
 
   

Which cardiac abnormality is characteristic of tuberous sclerosis?

Congenital rhabdomyomas (50%). These tumors are due to abnormal differentiation of embryonic myocardium into atypical Purkinje cells.  They are usually multiple and often clinically silent, although ventricular outflow obstruction may occur. They may regress later in childhood.

 
 
 
     
 

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