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DOI: 10.5935/abc.20180120 Brugada syndrome (BrS) was described by Pedro and Josep Brugada in 1992 as a new clinical entity characterized by specific electrocardiographic (ECG) changes, such as the patterns of right bundle-branch block and persistent ST-segment elevation in right precordial leads, associated with increased risk for sudden death.1 Brugada syndrome is an autosomal dominant channelopathy, with clinical manifestation at the age of 30 to 40 years, affecting mainly men. Currently, BrS is estimated to account for 12% of all sudden cardiac deaths and up to 20% of the sudden cardiac deaths in individuals with no structural heart disease.2 The real prevalence of BrS in the general population is difficult to establish, being estimated at 5 to 20 in every 10,000 individuals.2 Several genetic mutations have been associated with BrS, most of them related to the encoding of sodium channel proteins (INa), calcium channel proteins (ICa) or potassium channel proteins (usually Ito) of the sarcoplasmatic membrane.2-5 The usual clinical manifestation of BrS is arrhythmic syncope, nocturnal agonal respiration or sudden death secondary to polymorphic ventricular tachycardia (VT) or ventricular fibrillation (VF). The symptoms usually occur during sleep, at rest during the day or in situations with increased vagal tone, such as binge drinking or heavy meals. In addition, fever is a common trigger, mainly among children.2-5 The diagnosis and risk stratification of BrS are mainly based on clinical history and ECG pattern, which can generate controversy because of the incomplete penetrance of the channelopathy and the dynamic pattern of ECG manifestations.2-5 Although three ECG patterns have been described, the diagnosis of BrS can only be established in patients with type 1 ECG pattern (coved-type), characterized by a concave ST-segment elevation ≥ 2 mm in at least two right precordial leads (V1, V2) positioned on the 2nd, 3rd and 4th intercostal spaces, occurring spontaneously or after provocative drug test with the intravenous administration of class I antiarrhythmic drugs (ajmaline, flecainide or procainamide). The other ECG patterns (types 2 and 3) do not establish the diagnosis of BrS.2-5 In BrS risk stratification and treatment, individuals with history of resuscitated cardiac arrest (class I indication) and those with history of syncope and type 1 ECG pattern (class IIa indication) are at high risk for sudden death and have indication for implantable cardioverter-defibrillator (ICD) placement for secondary prevention. Several studies have reported that asymptomatic patients with type 1 ECG pattern only after infusion of class I antiarrhythmic drugs are at low risk for arrhythmic events during clinical follow-up.6-8 Thus, in those patients, ICD placement should be avoided because of the risk of complications, such as inappropriate shocks.3-5

Cohort of Patients Referred for Brugada Syndrome Investigation in an Electrophysiology Service - 19-Year Registry