What is the management for Brugada syndrome?

Management of Brugada Syndrome

The management of Brugada syndrome should be based on risk stratification, with ICD implantation recommended for high-risk patients who have spontaneous type 1 Brugada ECG pattern with cardiac arrest, sustained ventricular arrhythmias, or recent syncope presumed due to ventricular arrhythmias. 1, 2

Risk Stratification

Risk stratification is essential for determining appropriate management:

High-Risk Patients

• Spontaneous type 1 Brugada ECG pattern AND one of the following:
  • History of cardiac arrest
  • Documented sustained ventricular arrhythmias
  • Recent syncope presumed due to ventricular arrhythmias

Medium-Risk Patients

• Spontaneous type 1 Brugada ECG pattern without symptoms
• Ventricular fibrillation during programmed ventricular stimulation

Low-Risk Patients

• Only drug-induced type 1 ECG pattern without symptoms

Management Recommendations

1. Implantable Cardioverter Defibrillator (ICD)

• Class I recommendation (strong): ICD implantation for patients with Brugada syndrome with spontaneous type 1 ECG pattern AND cardiac arrest, sustained ventricular arrhythmias, or recent syncope presumed due to ventricular arrhythmias 1
• Only recommended if meaningful survival greater than 1 year is expected

2. Pharmacological Therapy

• For patients with recurrent ICD shocks for polymorphic VT: Quinidine is recommended (Class I) 1
• For patients with spontaneous type 1 ECG pattern and symptomatic ventricular arrhythmias who decline or are not candidates for ICD: Quinidine is recommended (Class I) 1, 2
• For acute management of electrical storms: Isoproterenol is recommended 2

3. Catheter Ablation

• For patients with recurrent ICD shocks for polymorphic VT: Catheter ablation is recommended (Class I) 1
• For patients with spontaneous type 1 ECG pattern and symptomatic ventricular arrhythmias who decline or are not candidates for ICD: Catheter ablation is recommended (Class I) 1
• Targets abnormal epicardial late activation areas in the right ventricle 2

4. Observation Without Therapy

• For asymptomatic patients with only inducible type 1 Brugada ECG pattern: Observation without therapy is recommended (Class I) 1
• For asymptomatic patients with early repolarization pattern: Observation without therapy is recommended 1

5. Diagnostic Testing

• For suspected Brugada syndrome without spontaneous type 1 ECG pattern: Pharmacological challenge using sodium channel blockers can be useful (Class IIa) 1
• For asymptomatic patients with spontaneous type 1 ECG pattern: Electrophysiological study with programmed ventricular stimulation may be considered for further risk stratification (Class IIb) 1

6. Genetic Testing

• For patients with suspected or established Brugada syndrome: Genetic counseling and testing may be useful to facilitate cascade screening of relatives (Class IIb) 1, 2
• SCN5A gene mutations are most common but genetic test results currently do not influence prognosis or treatment decisions 2

Lifestyle Modifications and Precautions

• Avoid medications that can induce ST-segment elevation (sodium channel blockers, certain calcium channel blockers, beta-blockers, tricyclic antidepressants) 2
• Avoid excessive alcohol intake and large meals 2
• Promptly treat fever with antipyretic drugs (fever can trigger arrhythmic events) 2, 3
• Check medication safety on www.brugadadrugs.org before taking new medications 2

Important Clinical Considerations

• Spontaneous type 1 ECG pattern and symptoms at diagnosis are the strongest predictors of life-threatening arrhythmias 3
• Even asymptomatic Brugada syndrome patients have an annual incidence of arrhythmic events of approximately 0.5% 2
• Fever is a significant trigger for cardiac arrest in Brugada syndrome patients, accounting for 27% of life-threatening arrhythmias in younger patients 3
• ICD implantation, while life-saving, carries a significant risk of complications, with major adverse events reported in 41% of pediatric patients 3

By following this structured approach to management based on risk stratification, clinicians can provide appropriate care for patients with Brugada syndrome while minimizing unnecessary interventions for low-risk individuals.