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Supraventricular tachycardia in a neonate with cardiac rhabdomyoma and tuberous sclerosis

Korean Journal of Pediatrics 2008;51(7):766-770.
Published online July 15, 2008.
Supraventricular tachycardia in a neonate with cardiac rhabdomyoma and tuberous sclerosis
In Kug Bang, Yeo Hyang Kim, Chun Soo Kim, Sang Lak Lee, Tae Chan Kwon
Department of Pediatrics, School of Medicine, Keimyung University, Daegu, Korea
상심실성 빈맥과 심장 종양으로 진단된 결절성 경화증 1예
방인국, 김여향, 김천수, 이상락, 권태찬
계명대학교 의과대학 소아과학교실
Correspondence: 
Yeo Hyang Kim, Email: kimyhped@hanmail.net
Abstract
Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.
Key Words: Tuberous sclerosis, Rhabdomyoma, Arrhythmia


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