INFORMATION ON TS HEART

MANIFESTATIONS

Up to two-third of individuals with Tuberous Sclerosis Complex have one or more cardiac rhabdomyomas, although few of these become symptomatic. Cardiac rhabdomyomas are considered hamartomas rather than a true neoplasm. The lesions tend to be multiple, and there is evidence that their frequency and size diminish with age.

Most of the patients with cardiac dysfunction due to cardiac rhabdomyomas present soon after birth with heart failure, although older children and adults occasionally present with cardiac arrhythmia.

DIAGNOSTIC SCREENING AND FOLLOW-UP

At diagnosis, an echocardiogram should be done in order to determine if cardiac rhabdomyomas are present. An EKG could identify an arrhythmia. Any symptoms should be followed by a cardiologist who is aware of the risks involved in patients with TSC.

TREATMENT

Cardiac rhabdomyoma of the heart is more likely to be the cause of heart failure in newborns or young children than in an adult with TSC. Most rhabdomyomas of the heart are asymptomatic, but should be treated by an experienced cardiologist if an arrhythmia or other cardiac problems are present. Cardiac arrhythmias have developed in children just after starting carbamazepine, but this does not seem to be a common complication.