Management of Brugada Syndrome
For patients with Brugada syndrome and a history of cardiac arrest or syncope, an implantable cardioverter-defibrillator (ICD) is the definitive treatment to prevent sudden cardiac death. 1
Risk-Based Treatment Algorithm
High-Risk Patients (Require ICD)
ICD implantation is mandatory for patients with: 1
- Survivors of cardiac arrest (annual event rate 13.5%) 1
- Documented spontaneous sustained ventricular tachycardia 1
- Spontaneous type 1 Brugada ECG pattern with syncope presumed arrhythmic (annual event rate 3.2%) 1
The ICD is the only treatment proven to prevent sudden cardiac death in Brugada syndrome. 1, 2 Research confirms that appropriate ICD therapy occurs almost exclusively in cardiac arrest survivors, with one multicenter study showing 8.4% of cardiac arrest survivors received appropriate shocks, while zero patients with syncope alone had arrhythmic events during follow-up. 3
Asymptomatic Patients
Observation without therapy is recommended for asymptomatic patients with only inducible type 1 Brugada pattern. 1, 2 The annual event rate in asymptomatic patients is only 1%, making aggressive intervention unwarranted. 1
Electrophysiological study with programmed ventricular stimulation may be considered for further risk stratification in asymptomatic patients with spontaneous type 1 ECG, but its predictive value remains controversial. 1
Alternative Therapies
Quinidine
Quinidine is recommended for patients who: 1
- Experience recurrent ICD shocks for polymorphic ventricular tachycardia (electrical storms) 1, 2
- Have contraindications to ICD or refuse device implantation 1, 2, 4
- Require treatment for supraventricular arrhythmias 1
Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation, though data confirming its ability to reduce sudden cardiac death risk are limited. 1, 2
Catheter Ablation
Epicardial catheter ablation over the anterior right ventricular outflow tract is recommended for: 1, 2
- Patients with electrical storms or repeated appropriate ICD shocks 1
- Patients with symptomatic ventricular arrhythmias who are not ICD candidates or decline ICD 1
This therapy may prevent electrical storms in patients with recurring episodes, though data require further confirmation. 1
Universal Lifestyle Modifications
All patients with Brugada syndrome must implement these lifestyle changes regardless of symptom status: 1, 2, 4
- Avoid drugs that induce ST-segment elevation in right precordial leads (consult www.brugadadrugs.org) 1, 4, 5
- Avoid excessive alcohol intake and large meals, which are known triggers for ventricular fibrillation 1, 2, 4
- Treat any fever immediately and aggressively with antipyretics, as fever precipitates cardiac arrest and accounts for 27% of life-threatening arrhythmic events 1, 2, 4
Diagnostic Considerations
For suspected Brugada syndrome without spontaneous type 1 ECG pattern, pharmacological challenge using a sodium channel blocker (ajmaline, procainamide, or pilsicainide) can be useful for diagnosis. 1, 2 This test must be performed in a monitored hospital setting. 5
Genetic counseling and testing may be useful to facilitate cascade screening of relatives, though results do not currently influence prognosis or treatment. 1
Critical Caveats
The complication rate with ICD therapy is substantial. Multicenter studies report overall complication rates of 28-32%, with inappropriate shocks occurring in 20-27% of patients. 3, 6 Inappropriate shocks are 2.5 times more frequent than appropriate ones. 6 Common complications include lead failure/dislodgment, T-wave oversensing, and psychiatric issues requiring assistance in 13.5% of patients. 3, 6
Spontaneous type 1 Brugada pattern carries worse prognosis than drug-induced pattern, making the distinction clinically important. 2
Males are affected 8-10 times more frequently than females, with mean age of ventricular fibrillation at 41±15 years, and arrhythmias typically occur during rest or sleep. 4