• Ali N Zaidi.
• Cardiology, Nationwide Children's Hospital, Division of Cardiovascular Medicine, The Ross Heart Hospital, The Ohio State University, Columbus, Ohio 43210, USA. ali.zaidi@osumc.edu
• Congenit Heart Dis. 2010 Nov 1;5(6):594-8.

AbstractBrugada syndrome is an arrhythmogenic disease characterized by a pattern of ST segment elevation in the right precordial leads on an electrocardiogram with an increased risk of sudden cardiac death. It is primarily reported in adults with limited data about the prevalence, prognosis, and long-term management in children. We describe a 10-year-old boy with a family history of sudden cardiac death, who had near syncope associated with a febrile illness. A screening electrocardiogram revealed ST segment elevation in the right precordial leads consistent with Type-1 Brugada syndrome. An electrocardiogram after recovery from his illness showed Type-2 "saddle-back" ST segment changes. An echocardiogram and a cardiac magnetic resonance imaging revealed a normal heart without myocardial fibrofatty infiltration, scar, or ischemia. A tilt-table test was negative. Implantable cardioverter-defibrillator placement remains the only effective treatment for patients with symptomatic Brugada syndrome; however, risk stratification of asymptomatic patients continues to remain a challenge. Although some investigators have reported the use of electrophysiological studies for distinguishing between high and low risk patients with Brugada syndrome, there are no precise predictors of risk for sudden cardiac death in pediatric patients. After careful discussion, this patient was considered intermediate to high risk for sudden cardiac death and had successful implantation of a transvenous defibrillator. Although Brugada syndrome is a rare diagnosis in the pediatric population, such patients should be referred for further evaluation and management.© 2010 Copyright the Author. Congenital Heart Disease © 2010 Wiley Periodicals, Inc.

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