Congenital heart rhabdomyomatosis is a rare condition that is associated in most cases with tuberous sclerosis, a genetic syndrome inherited with an autosomal dominant pattern. The last is caused by a mutation in tuberous sclerosis 1 or tuberous sclerosis 2 genes. We report a case of heart rhabdomyomatosis, associated with tuberous sclerosis. The lesion was discovered at 21st week of gestation of a 36-year-old woman.
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