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

Journal of the Korean Pediatric Society 2003;46(9):913-917.
Published online September 15, 2003.
A Case of Repeated Ventricular Tachycardia Caused by Cardiac Rhabdomyomas in an Infant with Tuberous Sclerosis
Hee Jung Joo, Min Seob Song, Tae Gyu Hwang, Chul Ho Kim
Department of Pediatrics, College of Medicine, Inje University, Busan, Korea
반복되는 심실성 빈맥을 보인 대동맥판하 횡문근종을 동반한 결절성 경화증 1례
주희정, 송민섭, 황태규, 김철호
인제대학교 의과대학 부산백병원 소아과
Correspondence: 
Min Seob Song, Email: msped@ijnc.inje.ac.kr
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.
Key Words: Tuberous sclerosis, Cardiac rhabdomyoma, Ventricular tachycardia


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