What is the primary management approach for patients diagnosed with Brugada syndrome?

Primary Management of Brugada Syndrome

All patients with Brugada syndrome must immediately implement strict lifestyle modifications, and ICD implantation is mandatory for survivors of cardiac arrest or those with documented sustained ventricular tachycardia, while quinidine serves as the primary alternative for patients who refuse or have contraindications to ICD. 1, 2

Universal Lifestyle Modifications (All Patients)

Every patient diagnosed with Brugada syndrome requires these non-negotiable lifestyle changes, regardless of symptom status:

• 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, 2
• Treat fever aggressively and immediately with antipyretics—fever is a critical trigger accounting for 27% of life-threatening arrhythmic events and can precipitate cardiac arrest 1, 2, 3
• Eliminate excessive alcohol intake and large meals, both established triggers for ventricular fibrillation 1, 2, 3

Risk-Stratified ICD Implantation

Class I Indication (Mandatory ICD):

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

Class IIa Indication (Strongly Consider ICD):

• Spontaneous type 1 ECG pattern with history of syncope presumed arrhythmic (3.2% annual event rate) 1, 2, 4
• The combination of spontaneous type 1 pattern and syncope carries substantially higher risk than either factor alone 5, 6

Class IIb Indication (May Consider ICD):

• Inducible VF during programmed ventricular stimulation with two or three extrastimuli at two sites 1
• However, no single clinical risk factor or EPS alone adequately identifies highest-risk patients—a multiparametric approach combining syncope, family history of sudden death, and positive EPS is superior 5
• Patients with spontaneous type 1 ECG and at least two risk factors (syncope, family history of sudden death, positive EPS) have up to 30% event rate 5

Low Risk (Observation):

• Asymptomatic patients with only drug-induced type 1 pattern have approximately 1% annual event rate and typically do not require ICD 1, 3

Quinidine Therapy

Quinidine is the primary pharmacologic alternative and should be considered in specific scenarios:

• Patients who qualify for ICD but refuse or have contraindications (Class IIa recommendation) 1, 2, 3
• Electrical storms or recurrent appropriate ICD shocks 1, 2, 3
• Patients requiring treatment for supraventricular arrhythmias 1
• Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation 2, 3

Catheter Ablation

• Epicardial substrate ablation may be considered for patients with electrical storms or repeated appropriate ICD shocks (Class IIb recommendation) 1, 3
• Recent data show 91% resolution of type 1 pattern post-ablation, with 12% recurrent VT/VF rate during follow-up 7
• Ablation targets the anterior right ventricular outflow tract epicardial substrate 3, 7

Critical Diagnostic Considerations

• Spontaneous type 1 pattern carries significantly worse prognosis than drug-induced pattern and is the strongest predictor of life-threatening arrhythmias 4, 3, 5, 6
• Type 1 pattern requires coved ST-segment elevation ≥2 mm in V1 and/or V2 (positioned in second, third, or fourth intercostal space) with negative T-waves 1, 4
• Use high electrode positioning in second and third intercostal spaces to improve detection 4

Common Pitfalls

• Do not rely on programmed electrical stimulation alone for risk stratification in asymptomatic patients—it has limited predictive value without clinical context 5, 8
• Males are affected 8-10 times more frequently than females, with mean age of ventricular fibrillation at 41 ± 15 years 2
• Arrhythmias typically occur during rest or sleep, not during exertion 1, 2
• Genetic testing (SCN5A mutations account for 20-30% of cases) does not currently influence prognosis or treatment decisions, though it facilitates family screening 1, 4, 3