Management and Treatment of Brugada Syndrome

Diagnosis

The European Society of Cardiology recommends that Brugada syndrome is diagnosed when ST-segment elevation with type 1 morphology ≥2 mm occurs in leads V1 and/or V2, either spontaneously or after provocative drug testing with sodium channel blockers such as ajmaline, flecainide, procainamide, or pilsicainide 1, 2, 4
High electrode positioning in the second and third intercostal spaces improves detection of the type 1 Brugada pattern, as recommended by the American College of Cardiology 5
The American Heart Association suggests that sodium channel blocker challenge should only be performed in monitored hospital settings and terminated immediately if ventricular arrhythmias, marked QRS widening, or type 1 pattern develops 5, 6

The European Heart Journal recommends that every patient with Brugada syndrome must adhere to strict lifestyle modifications, including avoiding all drugs that induce ST-segment elevation, treating any fever immediately and aggressively with antipyretics, and avoiding excessive alcohol intake and large meals 1, 2, 7, 3
The American College of Cardiology advises patients to avoid all drugs that induce ST-segment elevation, and to consult www.brugadadrugs.org for a comprehensive updated list of contraindicated medications 1, 5, 6
The European Society of Cardiology recommends treating any fever immediately and aggressively with antipyretics, as fever is a critical trigger accounting for 27% of life-threatening arrhythmic events 1, 5, 6

The European Heart Journal recommends ICD implantation for survivors of aborted cardiac arrest, with an annual event rate of 13.5% 1, 2, 3
The American College of Cardiology suggests ICD implantation for patients with documented spontaneous sustained ventricular tachycardia 1, 2, 7
The European Society of Cardiology recommends considering ICD implantation for patients with spontaneous type 1 ECG pattern and history of syncope presumed arrhythmic, with an annual event rate of 3.2% 1, 2, 3, 8

The European Heart Journal recommends considering quinidine for patients who qualify for ICD but have contraindications or refuse device implantation, for treatment of electrical storms, and for patients requiring treatment for supraventricular arrhythmias 1, 2, 7, 3
The American College of Cardiology suggests that quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation and suppresses the Ito current 7, 3
The European Society of Cardiology advises avoiding rifampicin as it decreases quinidine plasma concentrations and causes therapeutic failures 6

The European Heart Journal recommends considering catheter ablation of abnormal epicardial late activation areas in the right ventricular outflow tract for patients with electrical storms or repeated appropriate ICD shocks 1, 2, 7, 8
The American College of Cardiology suggests that small series show that spontaneous type 1 Brugada pattern may be eliminated in >75% of patients, with marked reduction in VT/VF recurrences 5
The European Society of Cardiology recommends that experience and follow-up remain limited, and ICD is still recommended for patients with prior syncope or sudden cardiac arrest even after ablation 5

The European Heart Journal recommends considering genetic testing, but notes that it does not influence risk stratification or treatment decisions 2, 7, 5
The American College of Cardiology suggests that the yield of genetic testing is approximately 20-30% in phenotype-positive patients, with SCN5A variants accounting for most genotype-positive cases 5
The European Society of Cardiology recommends that genotype status is not correlated with risk of adverse events, but identification of pathogenic mutations helps facilitate family screening and carrier recognition 5, 8