What is the primary treatment for Brugada syndrome?

From the Guidelines

The primary treatment for Brugada syndrome is the implantation of an implantable cardioverter-defibrillator (ICD), as recommended for patients who have survived a cardiac arrest, have documented ventricular arrhythmias, or are at high risk for sudden cardiac death based on clinical risk factors and electrophysiological testing. This recommendation is based on the most recent and highest quality study, which emphasizes the importance of ICD implantation in preventing sudden cardiac death in patients with Brugada syndrome 1.

Key Considerations

• The ICD continuously monitors heart rhythm and delivers an electrical shock when it detects potentially life-threatening arrhythmias, effectively preventing sudden cardiac death.
• For symptomatic patients experiencing arrhythmic storms, quinidine may be prescribed as an adjunctive therapy to suppress ventricular arrhythmias, as suggested by the 2018 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 1.
• Patients should avoid certain medications that can exacerbate the condition, avoid excessive alcohol consumption, promptly treat fevers with antipyretics, and undergo regular cardiac follow-ups.

Additional Recommendations

• Quinidine or catheter ablation may be considered in patients with Brugada syndrome who experience recurrent ICD shocks for polymorphic VT, as recommended by the 2018 AHA/ACC/HRS guideline 1.
• Genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives, as suggested by the 2018 AHA/ACC/HRS guideline 1.
• Lifestyle changes, such as avoidance of drugs that may induce ST-segment elevation in right precordial leads, avoidance of excessive alcohol intake and large meals, and prompt treatment of any fever with antipyretic drugs, are recommended in all patients with a diagnosis of Brugada syndrome, as stated in the 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 1.

From the Research

Treatment for Brugada Syndrome

The primary treatment for Brugada syndrome is the implantation of an implantable cardioverter-defibrillator (ICD) in patients who are at high risk of sudden cardiac death. This includes patients who have survived a cardiac arrest, those with a history of syncope, or individuals with a familial history of sudden death or inducible ventricular fibrillation at electrophysiologic study 2, 3, 4.

Indications for ICD Implantation

The indications for ICD implantation in Brugada syndrome patients include:

• A history of cardiac arrest
• Syncope
• Inducible ventricular fibrillation in asymptomatic patients
• A family history of sudden death
• Nonsustained ventricular arrhythmia

Efficacy and Complications of ICD Therapy

Studies have shown that ICD therapy is effective in treating potentially lethal arrhythmias in Brugada syndrome patients, with appropriate shocks occurring in 8-25% of patients during long-term follow-up 3, 4, 5. However, ICD therapy is also associated with a significant risk of device-related complications, including inappropriate shocks, lead failure, and device malfunction, which occur in 20-32% of patients 2, 3, 4.

Remote ICD Monitoring

Remote ICD monitoring has been shown to simplify follow-up and detect potential complications in Brugada syndrome patients, reducing the number of cardiology consultations and potentially preventing inappropriate shocks 6.

Risk Stratification

Risk stratification is important in identifying asymptomatic patients who may be at risk for arrhythmic events, and electrophysiologic studies may be helpful in this regard 4, 5. Further research in risk stratification can improve patient prognosis while avoiding unnecessary ICD implantation.

Key Findings

• ICD therapy is effective for primary and secondary prevention of sudden cardiac death in Brugada syndrome patients
• Appropriate shocks occur more frequently in patients presenting with ventricular tachycardia/ventricular fibrillation, but also occur in asymptomatic patients
• Inappropriate shocks and device-related complications are common in Brugada syndrome patients with ICDs
• Remote ICD monitoring can simplify follow-up and detect potential complications
• Risk stratification is important in identifying patients who may benefit from ICD implantation 2, 3, 4, 6, 5