What is the management and treatment for Brugada syndrome?

Management and Treatment of Brugada Syndrome

ICD implantation is the only proven therapy to prevent sudden cardiac death in Brugada syndrome and is mandatory for high-risk patients, including cardiac arrest survivors, those with documented sustained ventricular tachycardia, and patients with spontaneous type 1 ECG pattern plus syncope presumed arrhythmic. 1, 2

Risk Stratification Framework

The management approach depends critically on risk category, with annual arrhythmic event rates varying dramatically:

• Highest risk (13.5% annual event rate): Cardiac arrest survivors or documented sustained ventricular tachycardia 1, 2
• High risk (3.2% annual event rate): Spontaneous type 1 ECG pattern with syncope presumed arrhythmic 1, 3
• Lower risk (1% annual event rate): Asymptomatic patients with spontaneous type 1 pattern 1

The spontaneous type 1 pattern (coved ST-segment elevation ≥2 mm in V1 and/or V2 with negative T-waves) is the strongest predictor of life-threatening arrhythmias. 1, 3 Use high electrode positioning in the second and third intercostal spaces to improve detection. 3

Definitive Treatment Strategies

ICD Implantation (First-Line for High-Risk Patients)

ICD implantation is recommended (Class I) for patients with spontaneous type 1 ECG pattern and either cardiac arrest, documented sustained VT, or syncope presumed due to ventricular arrhythmia, provided meaningful survival greater than 1 year is expected. 1, 2, 3

• Admit immediately to monitored bed with continuous telemetry when syncope occurs, as it is presumed arrhythmic until proven otherwise 1
• Cardiology/electrophysiology consultation should occur during hospitalization for ICD evaluation 1
• Monitor for appropriate and inappropriate shocks post-implantation; consider quinidine or ablation for recurrent shocks 3

Common pitfall: ICD complications include inappropriate shocks, device malfunction, infection, and mental health problems, which must be discussed with patients. 4

Quinidine Therapy (Alternative or Adjunctive)

Quinidine is a Class I recommendation for patients who decline ICD or have contraindications, and Class IIa for those who qualify for ICD but refuse or cannot receive one. 1, 2, 3

• Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation 2
• Consider as first-line therapy for patients experiencing electrical storms 2

Catheter Ablation

• Right ventricular outflow tract ablation may be considered as an alternative option, particularly for recurrent ICD shocks 1, 3

Universal Lifestyle Modifications (All Patients)

All patients diagnosed with Brugada syndrome must implement strict lifestyle changes regardless of symptom status. 2

Medication Avoidance

• Immediately discontinue: Sodium channel blockers, QT-prolonging drugs, psychotropic agents, and specific anesthetic agents that trigger ventricular fibrillation 1, 2, 3
• Avoid cocaine 3

Trigger Management

• Treat fever immediately and aggressively with antipyretics, as fever precipitates cardiac arrest and accounts for 27% of life-threatening arrhythmic events 1, 2, 3
• Avoid excessive alcohol intake and large meals, which are known triggers for ventricular fibrillation 2, 3

Critical caveat: Arrhythmias typically occur during rest or sleep, not during exertion. 2

Asymptomatic Patient Management

For asymptomatic patients with spontaneous type 1 pattern (1% annual event rate), management is more nuanced:

• ICD placement can reduce mortality in select asymptomatic patients 4
• Risk factors favoring ICD include spontaneous type 1 pattern and inducibility of ventricular tachyarrhythmias during electrophysiological study 4
• Gender, family history of sudden cardiac death, and SCN5A mutation presence are NOT predictive of arrhythmic events 4
• Decision should weigh the 1% annual event rate against ICD complications 4

Family Screening and Genetic Counseling

• Genetic counseling and testing may be useful to facilitate cascade screening of relatives (Class IIb recommendation) 1, 3
• SCN5A mutations account for 20-30% of cases, though negative genetic testing does not exclude diagnosis 3
• Males are affected 8-10 times more frequently than females, with mean age of ventricular fibrillation at 41 ± 15 years 2

Special Considerations

• Prevalence is higher in Southeast Asia (1 in 1,000 to 1 in 10,000) compared to other populations 2
• Cardiac arrest can result in hypoxic brain damage requiring multidisciplinary rehabilitation 5
• ECG manifestations are dynamic and may only appear after sodium channel blocker administration or with fever 6