Management and Treatment of Brugada Syndrome
Immediate Lifestyle Modifications for All Patients
All patients diagnosed with Brugada syndrome must implement strict lifestyle changes to prevent sudden cardiac death, regardless of symptom status. 1
- Avoid all drugs that induce ST-segment elevation in right precordial leads (comprehensive list at www.brugadadrugs.org), including sodium channel blockers, certain psychotropic agents, and specific anesthetic drugs 1, 2, 3
- Avoid excessive alcohol intake and large meals, as these are known triggers for ventricular fibrillation 1
- Treat any fever immediately and aggressively with antipyretics, as fever is a critical trigger that can precipitate cardiac arrest and accounts for 27% of life-threatening arrhythmic events 1, 2, 4
ICD Implantation: The Only Proven Mortality-Reducing Therapy
ICD implantation is the only treatment proven to prevent sudden cardiac death in Brugada syndrome and is mandatory for high-risk patients. 1, 5
Class I Recommendations (Must Implant):
- Survivors of aborted cardiac arrest (annual event rate 13.5%) 1
- Documented spontaneous sustained ventricular tachycardia 1
Class IIa Recommendation (Should Strongly Consider):
- Spontaneous type 1 ECG pattern with history of syncope presumed arrhythmic (annual event rate 3.2%) 1, 2, 3
- The combination of spontaneous type 1 pattern and symptoms at diagnosis are the strongest predictors of life-threatening arrhythmias 3, 4
Class IIb Recommendation (May Consider):
- Inducible ventricular fibrillation during programmed ventricular stimulation with two or three extrastimuli at two sites 1
- Note: The prognostic value of electrophysiologic testing remains debated, with most studies failing to confirm positive or negative predictive value 1
Important Caveat:
- ICD complications occur in 41% of young patients, including inappropriate shocks, device malfunction, infection, and psychological issues 4, 6
- Asymptomatic patients with only drug-induced type 1 pattern have low annual event rates (1%) and observation without ICD is reasonable 1, 5
Quinidine: Alternative or Adjunctive Therapy
Quinidine should be considered as first-line therapy for patients who refuse ICD, have contraindications, or experience electrical storms. 1, 5
Class IIa Indications:
- Patients who qualify for ICD but refuse or have contraindications 1
- Treatment of supraventricular arrhythmias in Brugada patients 1
- Electrical storms or recurrent appropriate ICD shocks (in combination with isoproterenol) 1, 3
Evidence Base:
- Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation 1, 5
- In pediatric series, 8 of 11 patients treated with hydroquinidine remained asymptomatic 4
- Critical limitation: No data confirm quinidine's ability to reduce sudden cardiac death risk 1
Catheter Ablation: Emerging Rescue Therapy
Epicardial catheter ablation over the anterior right ventricular outflow tract may be considered for patients with electrical storms or repeated ICD shocks. 1, 5
- This is a Class IIb recommendation requiring confirmation before entering general clinical practice 1
- Consider as rescue therapy when quinidine and isoproterenol fail to control recurrent arrhythmias 3, 5
Risk Stratification Algorithm
Use this hierarchy to determine management intensity:
Highest Risk (13.5% annual event rate): Cardiac arrest survivors or documented sustained VT → ICD mandatory 1, 3
High Risk (3.2% annual event rate): Spontaneous type 1 ECG + syncope → ICD strongly recommended 1, 2, 3
Intermediate Risk: Spontaneous type 1 ECG without symptoms → Individualized decision, consider observation with strict lifestyle modifications 1
Lower Risk (1% annual event rate): Drug-induced type 1 pattern, asymptomatic → Observation with lifestyle modifications 1, 5
Genetic Testing and Family Screening
Genetic counseling and testing may be useful to facilitate cascade screening of relatives, though negative testing does not exclude diagnosis. 2, 3, 5
- SCN5A mutations account for 20-30% of cases 3, 7
- In one pediatric series, all 9 patients with life-threatening arrhythmias who underwent genetic testing were genotype positive, while 17 SCN5A-negative patients remained asymptomatic 4
- However, genetic results do not currently influence prognosis or treatment decisions 1
Special Populations and Considerations
Males are affected 8-10 times more frequently than females, with mean age of ventricular fibrillation at 41 ± 15 years. 1, 7