What is the primary treatment for Brugada syndrome?

Primary Treatment for Brugada Syndrome

The implantable cardioverter-defibrillator (ICD) is the only proven treatment to prevent sudden cardiac death in Brugada syndrome and represents the cornerstone of therapy for high-risk patients. 1, 2

Risk-Stratified Treatment Algorithm

Highest Risk: Mandatory ICD Implantation (Class I Recommendation)

ICD implantation is mandatory for patients who: 1

• Are survivors of aborted cardiac arrest (13.5% annual event rate) 3
• Have documented spontaneous sustained ventricular tachycardia 1

These patients have the highest mortality risk and ICD is non-negotiable. 2

High Risk: ICD Should Be Considered (Class IIa Recommendation)

ICD implantation should be strongly considered for patients with: 1

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

The combination of spontaneous (not drug-induced) type 1 pattern with syncope carries significantly worse prognosis. 2

Intermediate Risk: ICD May Be Considered (Class IIb Recommendation)

ICD may be considered for patients who: 1

• Develop ventricular fibrillation during programmed ventricular stimulation with 2-3 extrastimuli at two sites 1

The prognostic value of electrophysiologic testing remains debated, making this a weaker recommendation. 1

Low Risk: Observation Without ICD

Asymptomatic patients with only drug-inducible type 1 pattern should be observed without ICD (1% annual event rate). 2, 3

Universal Lifestyle Modifications (Class I Recommendation)

All patients with Brugada syndrome, regardless of risk category, must implement these lifestyle changes: 1, 3

• Avoid all drugs that induce ST-segment elevation (comprehensive list at www.brugadadrugs.org), including sodium channel blockers like flecainide, ajmaline, procainamide, certain psychotropics, and specific anesthetics 3, 4
• Treat any fever immediately and aggressively with antipyretics - fever is a critical trigger accounting for 27% of life-threatening arrhythmic events 3, 4
• Avoid excessive alcohol intake and large meals - both are known triggers for ventricular fibrillation 1, 3

Alternative Pharmacologic Therapy: Quinidine

Quinidine should be considered (Class IIa) for patients who: 1, 2

• Qualify for ICD but have contraindications or refuse the device 1, 3
• Experience electrical storms or recurrent ICD shocks 1, 2
• Require treatment for supraventricular arrhythmias 1

Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation and can prevent arrhythmic events. 2, 3 It represents the only pharmacologic option with evidence for risk reduction. 2

Rescue Therapy for Electrical Storms

For acute electrical storms, quinidine or isoproterenol should be considered (Class IIa). 1

Catheter ablation may be considered (Class IIb) for patients with: 1, 2

• History of electrical storms 1
• Repeated appropriate ICD shocks 1

Epicardial ablation over the anterior right ventricular outflow tract may prevent recurrent episodes. 2

Critical Clinical Pitfalls

The spontaneous type 1 Brugada pattern carries significantly worse prognosis than drug-induced pattern - this distinction is crucial for risk stratification. 2 Males are affected 8-10 times more frequently than females, with mean age of ventricular fibrillation at 41±15 years. 3 Arrhythmias characteristically occur during rest or sleep, not during exertion. 3

Risk stratification using combined factors is superior to single factors: patients with 2-3 risk factors (spontaneous type 1 ECG, syncope, inducible VF) experience significantly more arrhythmic events than those with 0-1 risk factors. 5