Management of Brugada Syndrome Based on ECG Findings
Implantable cardioverter-defibrillator (ICD) is the only treatment proven to reduce the risk of sudden cardiac death in Brugada syndrome and is strongly recommended for patients with characteristic ECG findings who have survived cardiac arrest or have documented spontaneous sustained ventricular tachycardia. 1
Diagnosis and ECG Characteristics
- Brugada syndrome is diagnosed by the presence of ST-segment elevation with type 1 morphology ≥2 mm in one or more leads among the right precordial leads V1 and/or V2 positioned in the second, third, or fourth intercostal space 1
- This ECG pattern may occur spontaneously or be induced by administration of sodium channel blockers (ajmaline, flecainide, procainamide, or pilsicainide) 1
- The characteristic type 1 Brugada pattern shows coved ST elevation in right precordial leads with negative T waves 1
- High ECG electrode positioning in the second and third interspaces improves detection of type 1 Brugada pattern 1
Risk Stratification
- Spontaneous type 1 Brugada ECG pattern carries a worse prognosis than drug-induced pattern 1
- Patients with syncope and spontaneous type 1 ECG pattern have a 6-fold higher risk of cardiac arrest than asymptomatic patients with spontaneous ECG pattern 1
- Annual incidence of arrhythmic events varies significantly: 13.5% per year in patients with history of sudden cardiac arrest, 3.2% per year in patients with syncope, and 1% per year in asymptomatic patients 1, 2
- Male gender and age (typically 30s-40s) are associated with higher risk, with VF occurring at a mean age of 41±15 years 1, 3
Management Algorithm
High-Risk Patients (Class I recommendation)
- ICD implantation is recommended for patients who: 1
- Are survivors of aborted cardiac arrest
- Have documented spontaneous sustained ventricular tachycardia
Intermediate-Risk Patients (Class IIa recommendation)
- ICD implantation should be considered for patients with: 1
- Spontaneous type 1 ECG pattern AND history of syncope
Management of Electrical Storms
- Quinidine or isoproterenol should be considered in patients experiencing electrical storms 1, 4
- Catheter ablation may be considered in patients with history of electrical storms or repeated appropriate ICD shocks 1
Low-Risk/Asymptomatic Patients
- For asymptomatic patients with only drug-induced type 1 pattern, observation without therapy is recommended 2, 5
- Programmed ventricular stimulation (PVS) has controversial value for risk stratification 1
Additional Management Recommendations
For All Patients with Brugada Syndrome
- Implement lifestyle modifications: 1
- Avoid drugs that may induce ST-segment elevation (www.brugadadrugs.org)
- Avoid excessive alcohol intake and large meals
- Prompt treatment of any fever with antipyretic drugs (fever can trigger arrhythmias)
Alternative Therapies
- Quinidine should be considered in patients who: 1, 2
- Qualify for an ICD but have contraindications or refuse it
- Require treatment for supraventricular arrhythmias
- Experience recurrent ICD shocks
Special Considerations
- Genetic testing may be useful for cascade screening of relatives but does not influence prognosis or treatment decisions 1
- SCN5A mutations account for approximately 20-30% of Brugada syndrome cases 1, 3
- Pediatric patients with both symptoms at diagnosis and spontaneous type 1 ECG pattern have shorter time to first life-threatening arrhythmic event 3
- ICD complications (inappropriate shocks, device malfunction, infection) should be considered when making treatment decisions, especially in young patients 3, 5