A pediatric case of Brugada syndrome diagnosed by fever-provoked ventricular tachycardia
Korean Journal of Pediatrics 2014³â 57±Ç 8È£ p.374 ~ p.378
±èÁö³ª(Kim Gee-Na) - Pusan National University School of Medicine Pusan National University Children¡¯s Hospital Department of Pediatrics
°æ¿¹Âù(Kyung Ye-Chan) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pediatrics
°À̼®(Kang I-Seok) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pediatrics
¼ÛÁø¿µ(Song Jin-Young) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pediatrics
ÇãÁØ(Huh June) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pediatrics
¿Â¿µ±Ù(On Young-Keun) - Sungkyunkwan University School of Medicine Department of Medicine
Abstract
Brugada syndrome is a rare channelopathy associated with the SCN5A gene that causes fatal ventricular arrhythmias. This case of Brugada syndrome, in which ventricular tachycardia (VT) was provoked by high fever, is the first report in a Korean child. The boy had retinoblastoma of his left eye diagnosed at 16 months of age. After chemotherapy, he contracted a catheter-related infection with a high fever up to 41¡ÆC leading to monomorphic VT. This was characterized as having right bundle branch block morphology, superior axis deviation, and a heart rate of 212/min. Direct current cardioversion recovered the VT to sinus rhythm after a lack of response to amiodarone and lidocaine. A second attack of VT that was not controlled by cardioversion, however, responded to lidocaine. The baseline electrocardiogram showed a long PR interval and QRS duration, and the patient¡¯s grandfather had a history of Brugada syndrome. A mutation in SCN5A was identified in this patient, his father, and his grandfather. The patient was treated with quinidine and followed up for 1 year.
Å°¿öµå
Brugada syndrome, Fever, Ventricular tachycardia, Child, Retinoblastoma
KMID :
1130320140570080374
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