What is the management and treatment for Brugada (Brugada syndrome) syndrome?

Management and Treatment of Brugada Syndrome

All patients with Brugada syndrome must implement strict lifestyle modifications regardless of symptoms, and ICD implantation is mandatory for survivors of cardiac arrest, those with documented sustained ventricular tachycardia, or those with spontaneous type 1 ECG pattern and syncope. 1, 2, 3

Diagnostic Confirmation

Before initiating treatment, confirm the diagnosis requires:

• Type 1 Brugada pattern (coved ST-elevation ≥2 mm) in leads V1 and/or V2 positioned in the 2nd, 3rd, or 4th intercostal space, either spontaneous or after sodium channel blocker provocation (ajmaline, flecainide, procainamide, or pilsicainide) 1
• Type 2 ECG pattern alone is NOT diagnostic and requires provocative drug testing to unmask type 1 pattern 4
• Verify correct lead placement by repeating ECG with V1-V2 in higher intercostal spaces to improve detection 4

Universal Lifestyle Modifications (All Patients)

These are mandatory for every diagnosed patient, regardless of risk category 1, 2, 3:

• Avoid all drugs that induce ST-elevation (comprehensive list at www.brugadadrugs.org) - includes sodium channel blockers, certain psychotropic agents, and specific anesthetics 1, 3
• Treat fever immediately and aggressively with antipyretics - fever triggers 27% of life-threatening arrhythmic events and can precipitate cardiac arrest 3, 5
• Avoid excessive alcohol intake and large meals - both are known triggers for ventricular fibrillation 1, 3
• Avoid cocaine and recreational drugs 4

Risk Stratification and ICD Decision Algorithm

Class I Indication (ICD Mandatory):

• Survivors of aborted cardiac arrest (annual event rate 13.5%) 1, 2, 3
• Documented spontaneous sustained ventricular tachycardia (annual event rate 13.5%) 1, 3

Class IIa Indication (ICD Should Be Considered):

• Spontaneous type 1 ECG pattern AND history of syncope (annual event rate 3.2%) 1, 2, 3
• Spontaneous type 1 pattern carries significantly worse prognosis than drug-induced pattern 2
• In pediatric patients, spontaneous type 1 ECG plus symptoms at diagnosis predicts shorter time to first life-threatening arrhythmia 5

Class IIb Indication (ICD May Be Considered):

• Inducible VF during programmed ventricular stimulation with 2-3 extrastimuli at two sites 1
• This remains controversial as prognostic value of PVS is debated 1

No ICD Indicated:

• Asymptomatic patients with only drug-induced type 1 pattern (annual event rate 1%) - observation without ICD therapy 2, 4
• These patients still require strict lifestyle modifications 4

Pharmacological Therapy: Quinidine

Quinidine should be considered (Class IIa) in the following scenarios 1, 2, 3:

• Patients who qualify for ICD but have contraindications or refuse device implantation 1, 3
• Treatment of electrical storms or recurrent ICD shocks 1, 2, 3
• Patients requiring treatment for supraventricular arrhythmias 1, 3
• Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation 2, 3
• In pediatric series, 8 of 11 patients (73%) treated with hydroquinidine remained asymptomatic 5

Catheter Ablation

Epicardial catheter ablation over anterior right ventricular outflow tract may be considered (Class IIb) for 1, 2, 3:

• Electrical storms (multiple episodes requiring intervention)
• Repeated appropriate ICD shocks despite other therapies
• This prevents recurrent episodes but does not replace ICD in high-risk patients 2

Acute Management of Electrical Storm

For patients experiencing electrical storm (multiple VF episodes):

• Quinidine or isoproterenol should be considered for acute suppression 1
• Aggressive fever control if present 3, 5
• Consider emergent catheter ablation if refractory 1, 2

Special Considerations

Pediatric Patients:

• Fever triggers 27% of life-threatening events - aggressive antipyretic use is critical 5
• ICD complications occur in 41% of pediatric patients, making quinidine particularly valuable in this population 5
• All patients with life-threatening arrhythmias in pediatric series were genotype-positive, while SCN5A-negative patients remained asymptomatic 5

Genetic Testing:

• Does not currently influence prognosis or treatment decisions 1
• May facilitate cascade screening of first-degree relatives 2, 4
• Negative genetic testing does not exclude diagnosis 4

Gender and Age:

• Males affected 8-10 times more frequently than females 3
• Mean age of ventricular fibrillation is 41 ± 15 years, but can occur at any age 1, 3
• Arrhythmias typically occur during rest or sleep 3

Common Pitfalls to Avoid

• Do not rely on single ECG - type 1 pattern can be transient and requires high precordial lead placement 4, 6
• Do not withhold antipyretics - fever is a critical modifiable trigger 3, 5
• Do not assume drug-induced pattern has same risk as spontaneous - spontaneous type 1 carries significantly higher risk 2
• Do not overlook ICD complications in young patients - 41% complication rate in pediatric series makes quinidine an important alternative 5