What is the management of Brugada syndrome?

From the Guidelines

The management of Brugada syndrome focuses on preventing life-threatening arrhythmias, with implantable cardioverter-defibrillators (ICDs) being the cornerstone of treatment for high-risk patients, as recommended by the 2018 AHA/ACC/HRS guideline 1. The diagnosis of Brugada syndrome is made in patients with ST-segment elevation with type 1 morphology ≥2 mm in one or more leads among the right precordial leads V1 and/or V2, occurring either spontaneously or after provocative drug test with intravenous administration of sodium channel blockers, as stated in the 2015 ESC guidelines 1.

Key Recommendations

• ICDs are strongly recommended for patients who have survived cardiac arrest, have documented ventricular arrhythmias, or have a spontaneous type 1 ECG pattern with syncope 1.
• For acute management of arrhythmic storms, intravenous isoproterenol or quinidine can be used to suppress ventricular arrhythmias, as suggested by the 2018 AHA/ACC/HRS guideline 1.
• Long-term pharmacological options include quinidine, which blocks the transient outward potassium current and can be used at doses of 300-600 mg twice daily for patients who refuse or cannot receive an ICD, as recommended by the 2015 ESC guidelines 1.
• Risk stratification is essential, considering factors such as male gender, spontaneous type 1 ECG pattern, history of syncope, and family history of sudden cardiac death, as stated in the 2015 ESC guidelines 1.
• Patients should avoid triggers that can precipitate arrhythmias, including certain medications, excessive alcohol consumption, and fever, which should be promptly treated with antipyretics, as recommended by the 2015 ESC guidelines 1.
• Genetic testing can help identify affected family members who require screening, though it doesn't typically alter management decisions for the index patient, as stated in the 2015 ESC guidelines 1.

Lifestyle Changes

• Avoidance of drugs that may induce ST-segment elevation in right precordial leads
• Avoidance of excessive alcohol intake and large meals
• Prompt treatment of any fever with antipyretic drugs, as recommended by the 2015 ESC guidelines 1.

Follow-up

Regular follow-up with a cardiac electrophysiologist is necessary to monitor for arrhythmias and adjust treatment as needed, as suggested by the 2018 AHA/ACC/HRS guideline 1.

From the Research

Management of Brugada Syndrome

The management of Brugada syndrome involves several strategies to prevent sudden cardiac death, including:

• Implantable cardioverter defibrillators (ICDs) for patients at high risk of sudden death, such as those with a history of cardiac arrest, syncope, or a familial history of sudden death 2, 3, 4
• Remote ICD monitoring to simplify follow-up and detect potential complications 3
• Antiarrhythmic medications, such as quinidine and isoproterenol, to prevent the induction of ventricular fibrillation 5
• Risk stratification using a multiparametric clinical and instrumental strategy to identify individuals at risk for sudden death 6

Implantable Cardioverter Defibrillators (ICDs)

ICDs are the primary treatment for preventing sudden cardiac death in patients with Brugada syndrome. Studies have shown that:

• ICDs are effective in preventing sudden death in patients with Brugada syndrome, particularly those with a history of cardiac arrest 2, 4
• However, ICDs are not without complications, including inappropriate shocks, lead failure, and device malfunction 2, 3, 4
• Remote ICD monitoring can help detect potential complications and simplify follow-up 3

Antiarrhythmic Medications

Antiarrhythmic medications, such as quinidine and isoproterenol, have been shown to be effective in preventing ventricular fibrillation in patients with Brugada syndrome:

• Quinidine has been established as an effective antiarrhythmic in Brugada syndrome, while isoproterenol has also shown promise 5
• The addition of drug therapy to prevent the induction of arrhythmias has been shown to reduce morbidity and mortality associated with Brugada syndrome 5

Risk Stratification

Risk stratification is critical in identifying individuals with Brugada syndrome who are at risk for sudden death:

• A multiparametric clinical and instrumental strategy is proposed for risk stratification, including genetic testing, ECG analysis, and clinical evaluation 6
• The aim of risk stratification is to identify individuals who would benefit from ICD implantation or other treatments to prevent sudden cardiac death 6