| A Case of Repeated Ventricular Tachycardia Caused by
Cardiac Rhabdomyomas in an Infant with Tuberous Sclerosis |
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Hee Jung Joo, Min Seob Song, Tae Gyu Hwang, Chul Ho Kim |
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Department of Pediatrics, College of Medicine, Inje University, Busan, Korea |
| 반복되는 심실성 빈맥을 보인 대동맥판하 횡문근종을 동반한 결절성 경화증 1례 |
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주희정, 송민섭, 황태규, 김철호 |
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인제대학교 의과대학 부산백병원 소아과 |
Correspondence:
Min Seob Song, Email: msped@ijnc.inje.ac.kr
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| Abstract |
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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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