Management of Brugada Syndrome
All patients with Brugada syndrome require immediate lifestyle modifications, and ICD implantation is the only proven therapy to prevent sudden cardiac death in high-risk patients, particularly cardiac arrest survivors and those with spontaneous type 1 ECG pattern plus syncope. 1, 2
Immediate Universal Interventions for All Patients
Every patient diagnosed with Brugada syndrome must implement these lifestyle changes regardless of symptoms:
- Avoid all drugs that induce ST-segment elevation in right precordial leads, including sodium channel blockers, certain psychotropic agents, and specific anesthetic drugs (comprehensive list at www.brugadadrugs.org) 1, 3
- Avoid excessive alcohol intake and large meals, as these are known triggers for ventricular fibrillation 1, 3
- Treat any fever immediately and aggressively with antipyretics, as fever is a critical trigger that precipitates cardiac arrest and accounts for 27% of life-threatening arrhythmic events 1, 3
Risk-Stratified ICD Implantation
The decision for ICD placement follows a clear hierarchy based on presentation and annual arrhythmic event rates:
Class I Recommendation (Mandatory ICD):
- Survivors of aborted cardiac arrest (annual event rate 13.5%) 1, 2
- Documented spontaneous sustained ventricular tachycardia 1
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 without prior arrest had appropriate therapy during 39-month follow-up 4. Another large multicenter study of 220 patients found an overall annual event rate of 2.6% over 3 years 5.
Class IIa Recommendation (Should Consider ICD):
- Spontaneous type 1 ECG pattern with history of syncope presumed arrhythmic (annual event rate 3.2%) 1, 2, 6
The spontaneous type 1 pattern carries significantly worse prognosis than drug-induced pattern 2. Syncope in Brugada syndrome is presumed arrhythmic until proven otherwise and warrants monitored admission with continuous telemetry 6.
Class IIb Recommendation (May Consider ICD):
- Inducible VF during programmed ventricular stimulation with two or three extrastimuli at two sites 1
However, the prognostic value of electrophysiologic testing remains controversial, as most clinical studies have not confirmed positive or negative predictive value for cardiac events 1. Research shows inducibility rates are similar between symptomatic (89.2%) and asymptomatic (93.3%) patients without predicting outcomes 4.
Observation Without ICD:
Pharmacologic Therapy with Quinidine
Quinidine is a Class IIa recommendation for specific clinical scenarios:
- Patients who qualify for ICD but refuse it or have contraindications 1, 3
- Patients requiring treatment for supraventricular arrhythmias 1
- Electrical storms or recurrent ICD shocks (quinidine or isoproterenol) 1, 2
Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation, though data confirming its ability to reduce 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) in patients with:
Critical Complications and Pitfalls
The complication rate with ICD therapy is substantial and must be weighed against benefits:
- Inappropriate shocks occur in 20-27% of patients (2.5 times more frequent than appropriate shocks in one large study), caused by lead failure/dislodgment, T-wave oversensing, sinus tachycardia, or supraventricular tachycardia 4, 5
- Overall device-related complication rate is 28-32%, with annual complication rate of 8.9% 4, 5
- Psychiatric assistance required in 13.5% of patients due to ICD-related complications, mostly inappropriate shocks 4
- Lead problems requiring reintervention occur in 18.6% of patients 4
- High defibrillation threshold (12%), high pacing threshold (27%), and low R-wave amplitude (15%) occur at implantation 5
These high complication rates are particularly relevant when considering ICD placement in asymptomatic patients or those with syncope alone, where the benefit-to-risk ratio is less favorable than in cardiac arrest survivors 4, 5, 7.
Genetic Testing and Family Screening
Genetic counseling and testing may be useful (Class IIb) to facilitate cascade screening of relatives, though results do not currently influence prognosis or treatment 1, 2, 6. At least 12 genes are associated with Brugada syndrome, but only SCN5A and CACN1Ac individually account for >5% of positively genotyped patients 1.