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

Management of Brugada Syndrome

All patients with Brugada syndrome require strict lifestyle modifications, and ICD implantation is the only proven therapy to prevent sudden cardiac death in high-risk patients, specifically those with prior cardiac arrest, documented sustained ventricular tachycardia, or spontaneous type 1 ECG pattern with syncope. 1

Universal Lifestyle Modifications (All Patients)

Every patient diagnosed with Brugada syndrome must implement these measures regardless of symptom status 1:

• Avoid all drugs that induce ST-segment elevation in right precordial leads (comprehensive list at www.brugadadrugs.org), including sodium channel blockers (flecainide, ajmaline, procainamide), certain psychotropics, and specific anesthetics 1, 2
• Avoid excessive alcohol intake and large meals, as these trigger ventricular fibrillation 1, 3
• Aggressively treat any fever with antipyretics immediately, as fever precipitates cardiac arrest and accounts for 27% of life-threatening arrhythmic events 1, 3, 4

Risk Stratification and ICD Indications

Class I Recommendations (ICD Mandatory)

ICD implantation is recommended for patients with 1:

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

Class IIa Recommendations (ICD Should Be Considered)

ICD implantation should be considered for 1:

• Spontaneous type 1 ECG pattern with history of syncope (annual event rate 3.2%) 1

The distinction between spontaneous versus drug-induced type 1 pattern is critical—spontaneous pattern carries significantly worse prognosis 1, 4.

Observation Without Therapy

Observation without treatment is recommended for 1:

• Asymptomatic patients with only drug-inducible type 1 pattern (annual event rate 1%) 1

Research data supports this conservative approach: in multicenter studies, no asymptomatic patients without prior cardiac arrest experienced arrhythmic events during follow-up averaging 39-45 months 5, 6.

Class IIb Recommendations (May Be Considered)

Electrophysiological study with programmed ventricular stimulation may be considered for further risk stratification in asymptomatic patients with spontaneous type 1 pattern, though its predictive value remains debated 1.

ICD may be considered in patients who develop ventricular fibrillation during programmed ventricular stimulation with two or three extrastimuli at two sites 1.

Alternative and Adjunctive Therapies

Quinidine Therapy

Quinidine should be considered (Class IIa) for 1, 7:

• Patients who qualify for ICD but have contraindications or refuse implantation 1
• Treatment of electrical storms or recurrent ICD shocks 1
• Patients requiring treatment for supraventricular arrhythmias 1

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

Catheter Ablation

Epicardial catheter ablation over the anterior right ventricular outflow tract may be considered (Class IIb) for patients with electrical storms or repeated appropriate ICD shocks, though data require further confirmation 1, 7.

Isoproterenol

Isoproterenol should be considered for acute treatment of electrical storms 1.

Diagnostic Considerations

Brugada syndrome is diagnosed when ST-segment elevation with type 1 morphology ≥2 mm occurs in one or more leads among right precordial leads V1 and/or V2 (positioned in second, third, or fourth intercostal space), either spontaneously or after provocative drug testing with sodium channel blockers 1.

Pharmacological challenge using sodium channel blockers (ajmaline, flecainide, procainamide, or pilsicainide) can be useful for diagnosis in suspected cases without spontaneous type 1 pattern 1, 7, 2.

Genetic Testing

Genetic counseling and testing may be useful to facilitate cascade screening of relatives, though results do not currently influence prognosis or treatment decisions 1. At least 12 genes are associated with Brugada syndrome, but only SCN5A and CACN1Ac individually account for >5% of positively genotyped patients 1.

In pediatric populations, all patients with life-threatening arrhythmias were genotype positive, whereas SCN5A-negative patients remained asymptomatic, suggesting potential prognostic value in young patients 4.

ICD Complications and Considerations

The complication rate with ICD therapy is substantial and must be weighed against benefits 5, 6:

• Inappropriate shocks occur in 20-27% of patients (2.5 times more frequent than appropriate shocks), caused by lead failure/dislodgment, T-wave oversensing, sinus tachycardia, or supraventricular tachycardia 5, 6
• Overall complication rate is 28-32%, including lead problems requiring reintervention in 18.6% 5, 6
• Annual event rate for appropriate ICD therapy is only 2.6% in mixed-risk populations 6
• Psychiatric assistance required in 13.5% due to device-related complications 5

In pediatric patients, major adverse ICD events occurred in 41%, making aggressive use of antipyretics and quinidine particularly important in this population 4.

Common Pitfalls

• Do not use programmed ventricular stimulation as the sole criterion for ICD implantation in asymptomatic patients—its predictive value is not confirmed 1
• Do not overlook fever as a critical trigger—aggressive antipyretic therapy is essential and may prevent 27% of life-threatening events 3, 4
• Do not equate drug-induced and spontaneous type 1 patterns—spontaneous pattern carries significantly higher risk 1, 4
• Do not perform sodium channel blocker challenge outside monitored hospital settings—terminate immediately if ventricular arrhythmias or marked QRS widening develop 2