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

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.

반복되는 심실성 빈맥을 보인 대동맥판하 횡문근종을 동반한 결절성 경화증 1례

저자들은 응급 전기적 심율동 전환 및 지속적 항 부정맥 약물치료에도 불구하고 반복되는 심실성 빈맥을 보인 결절성 경화증 환아에서 심장 횡문근종의 외과적 절제 후 효과적으로 부정맥이 조절된 1례를 치험하였기에 보고하는 바이다.