What are the management and treatment options for Brugada syndrome?

Management and Treatment of Brugada Syndrome

Implantable cardioverter defibrillator (ICD) is the only treatment proven to reduce mortality in high-risk Brugada syndrome patients, and should be recommended for all patients with previous cardiac arrest, documented ventricular tachycardia, or syncope with spontaneous type 1 ECG pattern. 1

Diagnosis and Risk Stratification

Brugada syndrome is diagnosed when a patient presents with:

• ST-segment elevation with type 1 morphology ≥2 mm in one or more right precordial leads (V1, V2) positioned in the 2nd, 3rd, or 4th intercostal space
• This pattern may occur spontaneously or after provocative drug testing with sodium channel blockers 1

Risk stratification is critical for determining management:

Risk Category	Clinical Features	Annual Event Rate
High Risk	Previous cardiac arrest or sustained VT	13.5% per year
Intermediate Risk	Syncope with spontaneous type 1 ECG	3.2% per year
Low Risk	Asymptomatic	1% per year

Treatment Algorithm

1. High-Risk Patients

• ICD implantation is strongly recommended (Class I) for:
  • Survivors of aborted cardiac arrest
  • Patients with documented spontaneous sustained ventricular tachycardia 1
  • Expected meaningful survival >1 year 1

2. Intermediate-Risk Patients

• ICD implantation should be considered (Class IIa) for:
  • Patients with spontaneous type 1 ECG pattern and history of syncope 1

3. Low-Risk Patients

• Observation without therapy is recommended (Class I) for:
  • Asymptomatic patients with only inducible type 1 Brugada ECG pattern 1
  • ICD may be considered (Class IIb) in patients who develop VF during programmed ventricular stimulation with 2-3 extrastimuli 1

4. Management of Electrical Storms/Recurrent ICD Shocks

• Quinidine or isoproterenol should be considered (Class IIa) for:
  • Patients experiencing electrical storms 1
  • Patients with recurrent ICD shocks for polymorphic VT 1

5. Alternative Therapies

• Quinidine should be considered (Class IIa) for:

  • Patients who qualify for ICD but have contraindications or refuse it
  • Patients requiring treatment for supraventricular arrhythmias 1

• Catheter ablation may be considered (Class IIb) for:

  • Patients with history of electrical storms
  • Patients with repeated appropriate ICD shocks 1

Lifestyle Modifications

All patients with Brugada syndrome should follow these recommendations:

• Avoid drugs that may induce ST-segment elevation in right precordial leads (consult www.brugadadrugs.org)
• Avoid excessive alcohol intake
• Avoid large meals
• Promptly treat any fever with antipyretic drugs 1

Special Considerations

ICD Complications

The complication rate with ICDs in Brugada syndrome is significant (28-32%), including:

• Inappropriate shocks (20-27% of patients) due to lead failure, T-wave oversensing, sinus tachycardia, or SVT 2, 3
• Lead problems requiring reintervention (10-19%) 2
• Psychological impact (13.5% requiring psychiatric assistance) 2

Pediatric Patients

In young patients with Brugada syndrome:

• Spontaneous type 1 ECG pattern and symptoms at diagnosis are strong predictors of life-threatening arrhythmias
• Fever triggers approximately 27% of life-threatening arrhythmic events
• Aggressive use of antipyretics is important in prevention 4

Remote Monitoring

Remote ICD monitoring in Brugada syndrome patients can:

• Decrease outpatient consultations
• Help prevent inappropriate shocks through early detection of device issues
• Allow prompt reprogramming when needed 5

Genetic Testing

• Genetic testing may be useful to facilitate cascade screening of relatives (Class IIb) 1
• SCN5A mutations account for only about 5% of genotype-positive patients
• Results of genetic screening do not currently influence prognosis or treatment decisions 1

By following this evidence-based approach to management, clinicians can optimize outcomes for patients with Brugada syndrome while minimizing complications from unnecessary interventions.