Nguyen Timothy, Smythe John, Baranchuk Adrian
Division of Cardiology, Kingston General Hospital, Queen's University, Canada.
Cardiol Young. 2011 Oct;21(5):591-4. doi: 10.1017/S1047951111000424. Epub 2011 May 4.
Brugada syndrome is a channelopathy characterised electrocardiographically by distinctive coved ST-segment elevation in the right precordial leads and is associated with a predisposition for sudden death secondary to ventricular arrhythmias in otherwise healthy patients. Previously known as Brugada-like patterns, Brugada phenocopies include agents and conditions that mimic true Brugada syndrome, presenting with an acquired Brugada Type-1 ECG pattern. We describe the first reported case of a 17-month-old female with an asymptomatic rhabdomyoma of the interventricular septum that presented as a Brugada phenocopy.