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℃ 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.