Brugada Syndrome: Primary Treatment
An implantable cardioverter-defibrillator (ICD) is the only proven therapy to prevent sudden cardiac death in Brugada syndrome patients with cardiac arrest or syncope, and should be implanted in all such patients with meaningful survival greater than 1 year expected. 1, 2
High-Risk Patients Requiring ICD
The following patients with Brugada syndrome have Class I (strongest) recommendations for ICD implantation:
- Cardiac arrest survivors – ICD is mandatory regardless of ECG pattern 1, 2
- Spontaneous type 1 ECG pattern with syncope presumed due to ventricular arrhythmia – ICD should be implanted as syncope carries a 3.2% annual arrhythmic event rate compared to 13.5% in cardiac arrest survivors 1, 3
- Documented spontaneous sustained ventricular tachycardia – ICD is required 1
The spontaneous type 1 Brugada pattern (coved ST-segment elevation ≥2 mm in V1/V2 with negative T-waves) carries significantly worse prognosis than drug-induced pattern and is critical for risk stratification. 2, 3
Alternative Therapies for ICD-Ineligible Patients
Quinidine is the recommended alternative for patients who decline ICD or have contraindications, as it suppresses the Ito current and reduces ventricular fibrillation inducibility during programmed ventricular stimulation. 1, 2 This is a Class I recommendation from both European and American guidelines. 1
Catheter ablation of the right ventricular outflow tract is also recommended as an alternative option for ICD-ineligible patients. 1, 2, 3
Management of Recurrent ICD Shocks
For patients experiencing electrical storms or repeated appropriate ICD shocks, intensification of therapy with quinidine or catheter ablation is mandatory (Class I recommendation). 1, 2 Epicardial catheter ablation over the anterior right ventricular outflow tract may prevent electrical storms in patients with recurring episodes. 1, 2
Asymptomatic Patients
Observation without therapy is recommended for asymptomatic patients with only inducible type 1 Brugada pattern, as they have only a 1% annual arrhythmic event rate. 1, 2
Electrophysiological study with programmed ventricular stimulation may be considered (Class IIb) for further risk stratification in asymptomatic patients with spontaneous type 1 pattern, though its prognostic value remains debated. 1
Essential Lifestyle Modifications for All Patients
Regardless of treatment strategy, all Brugada syndrome patients must implement these lifestyle changes (Class I recommendation):
- Strict avoidance of contraindicated medications including sodium channel blockers (flecainide, ajmaline, procainamide), certain psychotropics, and QT-prolonging agents – refer patients to www.brugadadrugs.org 1, 2, 4
- Aggressive treatment of fever with antipyretics as fever can acutely precipitate cardiac arrest 1, 2, 3, 4
- Avoidance of excessive alcohol intake and large meals which can trigger ventricular fibrillation 1, 2, 4
Important Caveats About ICD Therapy
While ICD is life-saving, device-related complications are substantial in Brugada syndrome patients:
- Inappropriate shocks occur in 20-27% of patients, often 2.5 times more frequent than appropriate shocks 5, 6, 7
- Overall complication rate reaches 28-32%, including lead failures, T-wave oversensing, and device infections 5, 6, 7
- Subcutaneous ICD (S-ICD) has emerged as a promising alternative with appropriate shocks in 3% and inappropriate shocks in 7% during median 52-month follow-up, though careful screening including exercise testing is essential 8, 9
Shock zone programming at 250 beats/min and ensuring more than 1 suitable vector on screening are protective factors against inappropriate shocks. 9