Extract

Brugada syndrome is increasingly recognized as a disease entity associated with sudden cardiac death in generally relatively young individuals without structural heart disease. Right precordial ST-segment elevation is considered the hallmark of the Brugada ECG, but discrete prolongation of various conduction parameters is also frequently encountered. It is well known that the ECG is variable from day to day and varies between the three defined ECG types (types 1, 2, and 3).1,2 Type 1, i.e. the ‘coved-type’ ST-segment, is mandatory for the diagnosis. Type 2 is referred to as the ‘saddle-back’ type and type 3, a right precordial saddle-back type ST-segment with minor deviation from the isoelectric segment, cannot be distinguished from normal. When associated with documented (or inducible) ventricular arrhythmias or premature sudden cardiac death or diagnostic ECGs in family members or nocturnal agonal respiration, Brugada syndrome is diagnosed.1,2 In the absence of a baseline type 1 ECG, exposure to sodium channel blockers (i.e. flecainide and ajmaline i.v.) can unmask the required ECG. A genetic diagnosis, encountered by the identification of SCN5a mutations in up to 30% of patients, is not mandatory for the diagnosis.

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