Brugada Syndrome Management

Diagnosis and Risk Stratification

• Brugada syndrome diagnosis is based on documentation of a spontaneous or drug-induced type 1 Brugada electrocardiographic pattern, with pharmacological challenge using sodium channel blockers being useful when the spontaneous pattern is absent, as recommended by the American College of Cardiology and the European Society of Cardiology 1, 2, 3
• The use of ECG electrodes positioned in the 2nd and 3rd intercostal spaces improves the detection of the type 1 pattern 4
• Recent syncope presumed due to ventricular arrhythmia is a risk factor for Brugada syndrome, as noted by the American College of Cardiology and the American Heart Association 2, 3
• Programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification in asymptomatic patients with spontaneous type 1 Brugada pattern, as recommended by the American College of Cardiology and the American Heart Association 2, 3
• The European Society of Cardiology classifies Brugada syndrome patients into high-risk, medium-risk, and low-risk categories, with high-risk patients including those who have survived cardiac arrest, have documented spontaneous sustained ventricular tachycardia, or have a spontaneous type 1 ECG pattern with a history of syncope 5

Treatment and Management

• The American College of Cardiology recommends implantation of an automatic implantable cardioverter-defibrillator (AICD) in patients with spontaneous type 1 ECG pattern and syncope or cardiac arrest, if a significant survival of more than one year is expected 3
• ICD implantation is recommended as a Class I indication for patients who have survived cardiac arrest or have documented spontaneous sustained ventricular tachycardia, and as a Class IIa indication for patients with a spontaneous type 1 ECG pattern and a history of syncope 5, 4
• Quinidine or catheter ablation can be used to treat patients with spontaneous type 1 ECG pattern and symptomatic ventricular arrhythmias who are not candidates or refuse an AICD 3, 4
• Quinidine is the most effective pharmacological agent for Brugada syndrome patients with VT, and it may be used as an alternative when ICD is contraindicated or refused, as recommended by the European Heart Society guidelines 5
• Isoproterenol is recommended for acute management of electrical storms or repetitive VT episodes, with an initial bolus injection and continuous infusion, according to the European Heart Society guidelines 5
• Catheter ablation may be considered in patients with history of electrical storms or repeated appropriate ICD shocks (Class IIb), as it can suppress recurrent ventricular arrhythmias, according to the European Heart Society guidelines 5

Genetic Testing

• Genetic testing can be useful for facilitating cascade screening of family members, as recommended by the American College of Cardiology 3
• The yield of genetic testing in phenotypically positive patients is approximately 20-30% 4
• Genetic testing for SCN5A gene mutations may be considered, although current genetic test results do not influence prognosis or treatment decisions in Brugada syndrome patients 5
• Genotypic status is not correlated with the risk of adverse events 4

Trigger Avoidance

• Certain psychotropic medications, anesthetics, cocaine, excessive alcohol consumption, and fever should be avoided as they can trigger arrhythmic events, as recommended by the European Heart Society guidelines 4
• Patients should avoid certain medications that can induce ST-segment elevation in the right precordial leads, including:

• Beta-blockers should be avoided in Brugada syndrome patients as they can be potentially harmful, unlike in other channelopathies 5
• Patients with Brugada syndrome should avoid medications that can induce ST-segment elevation, excessive alcohol intake, and large meals, and promptly treat fever with antipyretic drugs, as recommended by the European Heart Society guidelines 5
• Fever can acutely increase the risk of cardiac arrest in Brugada syndrome patients 6
• Even asymptomatic Brugada syndrome patients are at significant risk of arrhythmic events (annual incidence of 0.5%) 6
• Spontaneous type 1 EKG pattern and symptoms at diagnosis are predictors of life-threatening arrhythmias 6