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KMID : 1130320080510070766
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Korean Journal of Pediatrics
2008 Volume.51 No. 7 p.766 ~ p.770
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Supraventricular tachycardia in a neonate with cardiac rhabdomyoma and tuberous sclerosis
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Bang In-Kug
Kim Yeo-Hyang
Kim Chun-Soo
Lee Sang-Lak
Kwon Tae-Chan
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Abstract
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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.
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KEYWORD
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Tuberous sclerosis, Rhabdomyoma, Arrhythmia
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