Korean J Pediatr.  2014 Aug;57(8):374-378.

A pediatric case of Brugada syndrome diagnosed by fever-provoked ventricular tachycardia

• Kim G
• Kyung YC
• Kang IS
• Song J
• Huh J
• On YK

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 41degrees C 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.

Keyword

Brugada syndrome; Fever; Ventricular tachycardia; Child; Retinoblastoma

MeSH Terms

Amiodarone
Arrhythmias, Cardiac
Axis, Cervical Vertebra
Brugada Syndrome*
Bundle-Branch Block
Catheter-Related Infections
Channelopathies
Child
Drug Therapy
Electric Countershock
Electrocardiography
Fathers
Fever
Heart Rate
Humans
Lidocaine
Male
Quinidine
Retinoblastoma
Tachycardia, Ventricular*
Amiodarone
Lidocaine
Quinidine