Key result
A 3-year-old male presenting with syncope was diagnosed with Brugada syndrome after a procainamide challenge unmasked classic EKG findings, leading to successful ICD placement.
Why the study?
Brugada syndrome typically presents in adulthood and is extremely rare in childhood, especially when symptomatic.
Case Report (n=1)
This case highlights the rare presentation of symptomatic Brugada syndrome in early childhood and the utility of a sodium channel blocker challenge when initial EKG findings are equivocal.
May support sodium channel blocker challenge in equivocal pediatric syncope; hypothesis-generating and should not yet change practice.
Syncope is a common presenting complaint in pediatric emergency departments. The differential diagnosis of syncope is vast, including various neurological and cardiac conditions. Brugada syndrome (BrS) is a primary inherited arrhythmia which can present with syncope but can lead to sudden cardiac deaths due to ventricular fi brillation. BrS is a rare genetic condition, often diagnosed in the adulthood. BrS presenting in childhood, especially symptomatic BrS, is extremely rare. We present a case of a previously healthy 3-year-old male who presented to the emergency department with syncope and was later diagnosed with BrS.
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Gupta et al. (2020) conducted a case report in Syncope / Brugada syndrome (n=1). Procainamide challenge was evaluated. A 3-year-old male presenting with syncope was diagnosed with Brugada syndrome after a procainamide challenge unmasked classic EKG findings, leading to successful ICD placement.
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