What is the management for Brugada syndrome?

Management of Brugada Syndrome

Mandatory Lifestyle Modifications for All Patients

All patients diagnosed with Brugada syndrome must implement strict lifestyle changes regardless of symptom status. 1, 2, 3

• Avoid all drugs that induce ST-segment elevation in right precordial leads, including sodium channel blockers, certain psychotropic agents, and specific anesthetic drugs (comprehensive list at www.brugadadrugs.org). 1, 3
• Avoid excessive alcohol intake and large meals, as these are known triggers for ventricular fibrillation. 1, 3
• Treat any fever immediately and aggressively with antipyretic drugs, as fever is a critical trigger that precipitates cardiac arrest and accounts for 27% of life-threatening arrhythmic events. 1, 3

ICD Implantation: The Only Proven Life-Saving Therapy

ICD implantation is the only treatment proven to prevent sudden cardiac death in Brugada syndrome and represents the cornerstone of therapy. 1, 2, 3

Class I Indications (Mandatory ICD):

• Survivors of aborted cardiac arrest (annual event rate 13.5%). 1, 3, 4
• Documented spontaneous sustained ventricular tachycardia. 1, 4

Class IIa Indications (Should Strongly Consider ICD):

• Spontaneous type 1 ECG pattern AND history of syncope (annual event rate 3.2%). 1, 3, 4

Class IIb Indications (May Consider ICD):

• Inducible VF during programmed ventricular stimulation with two or three extrastimuli at two sites (though prognostic value is debated). 1

Important Caveats About ICD Therapy:

• Asymptomatic patients with only drug-induced type 1 pattern have low risk (1% annual event rate) and do not require ICD. 1, 3, 4
• Spontaneous type 1 pattern carries significantly worse prognosis than drug-induced pattern. 2, 4
• Complication rates are substantial: inappropriate shocks occur in 7-20% of patients (2.5 times more frequent than appropriate shocks in some series), with overall device-related complications of 4-28%. 5, 6, 7
• Subcutaneous ICD (S-ICD) is emerging as a viable alternative to transvenous ICD, with appropriate shock delivery in 3% of patients and 90-100% first-shock success rate, though careful screening during exercise and after drug provocation is essential. 8, 5

Quinidine Therapy: Alternative and Adjunctive Treatment

Quinidine should be considered as first-line therapy for specific patient populations. 1, 2, 3

Class IIa Indications for Quinidine:

• Patients who qualify for ICD but refuse it or have contraindications. 1, 3
• Patients requiring treatment for supraventricular arrhythmias. 1
• Patients experiencing electrical storms or recurrent ICD shocks. 1, 2

Mechanism and Evidence:

• Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation, though data confirming reduction in sudden cardiac death risk are limited. 1, 3

Catheter Ablation: For Refractory Cases

Catheter ablation may be considered in patients with electrical storms or repeated appropriate ICD shocks (Class IIb). 1, 2

• Epicardial ablation over the anterior right ventricular outflow tract may prevent electrical storms in patients with recurring episodes. 1, 2
• This represents an emerging therapy with limited long-term data. 1

Risk Stratification Algorithm

Use the following hierarchy to determine management intensity based on annual arrhythmic event rates: 1, 3, 4

1. Highest Risk (13.5% annual event rate): Cardiac arrest survivors or documented sustained VT → Mandatory ICD
2. High Risk (3.2% annual event rate): Spontaneous type 1 ECG + syncope → Strongly consider ICD
3. Low Risk (1% annual event rate): Asymptomatic with spontaneous type 1 ECG → Observation with lifestyle modifications
4. Very Low Risk: Drug-induced type 1 pattern only → Observation with lifestyle modifications

Special Considerations

Diagnostic Nuances:

• The type 1 Brugada ECG pattern is transient and variable, requiring serial ECGs as a single normal ECG does not exclude diagnosis. 4
• Diagnosis requires ST-segment elevation with type 1 morphology ≥2 mm in V1 and/or V2 positioned in the second, third, or fourth intercostal space, occurring spontaneously or after sodium channel blocker challenge. 1, 4
• High electrode positioning in the second and third interspaces improves detection. 4

Population Characteristics:

• Males are affected 8-10 times more frequently than females, with mean age of ventricular fibrillation at 41 ± 15 years. 1, 3
• Arrhythmias typically occur during rest or sleep. 1, 3
• Prevalence is higher in Southeast Asia (1 in 1,000 to 1 in 10,000). 1, 3

Genetic Testing:

• Genetic testing may be useful for cascade screening of relatives but does not influence prognosis or treatment decisions. 1, 4
• SCN5A mutations account for approximately 20-30% of cases. 4

Critical Pitfall to Avoid:

• Programmed ventricular stimulation has limited predictive value and should not be the sole basis for ICD decisions in asymptomatic patients, as both positive and negative results have poor predictive accuracy for future events. 1