Brugada Syndrome is a genetic disorder that results in sudden cardiac death from polymorphic ventricular tachycardia or ventricular fibrillation in the setting of a structurally normal heart. This is most commonly from a mutation in the sodium channel ― gene SCN5A. Unlike other genetic syndromes that result in sudden cardiac death, the QT interval is normal in Brugada Syndrome.
There are three types of ECG findings in patients with Brugada Syndrome:
Type I: Lead V1 has a “coved” ST segment elevation of at least 2 millimeters, followed by a negative T wave.
Type II: There is a “saddleback” appearance of the ST segment in lead V1 with ST segment elevation of at least 2 millimeters; this can be present in normal individuals as well.
Type III: Features of type I (coved) or type II (saddleback) with less than 2 millimeters of ST segment elevation.
These ECG changes can be provoked in the electrophysiology lab by infusing ajmaline or procainamide.
The treatment for Brugada Syndrome is an implantable cardioverter defibrillator, or ICD.