Perioperative management of a 7-year-old child with Brugada syndrome

Brugada syndrome results from abnormalities in the myocardial transmembrane conduction of sodium, resulting in the characteristic electrocardiographic changes of ST segment elevation in the precordial leads and incomplete right bundle branch block in an otherwise structurally normal heart. Affected patients are frequently asymptomatic until their presentation with potentially lethal arrhythmias including ventricular fibrillation. The youngest reported patient with Brugada syndrome to undergo anesthetic management is presented in this article; the pathophysiology of the syndrome is reviewed, and its perioperative implications are discussed.