J Korean Pediatr Soc.  2003 Sep;46(9):913-917.

A Case of Repeated Ventricular Tachycardia Caused by Cardiac Rhabdomyomas in an Infant with Tuberous Sclerosis

• Joo HJ
• Song MS
• Hwang TG
• Kim CH

Abstract

Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained arrythmia in spite of intravenous amiodarone therapy. The pathologic examination confirmed the diagnosis of rhabdomyoma. The patient had no more arrythmia during the 14 month follow up period. Although cardiac rhabdomyomas may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrythmia.

Keyword

Tuberous sclerosis; Cardiac rhabdomyoma; Ventricular tachycardia

MeSH Terms

Amiodarone
Aortic Valve
Arrhythmias, Cardiac
Diagnosis
Follow-Up Studies
Humans
Infant*
Rhabdomyoma*
Status Epilepticus
Tachycardia, Ventricular*
Tuberous Sclerosis*
Amiodarone