What is the management and treatment for a patient with Brugada syndrome?

Management and Treatment of Brugada Syndrome

All patients diagnosed with Brugada syndrome must implement strict lifestyle modifications immediately, and ICD implantation is the only proven therapy to prevent sudden cardiac death in high-risk patients. 1, 2

Diagnosis

Brugada syndrome is diagnosed when ST-segment elevation with type 1 morphology ≥2 mm occurs in leads V1 and/or V2 (positioned in the second, third, or fourth intercostal space), either spontaneously or after provocative drug testing with sodium channel blockers such as ajmaline, flecainide, procainamide, or pilsicainide. 1

• High electrode positioning in the second and third intercostal spaces improves detection of the type 1 Brugada pattern 1
• Sodium channel blocker challenge should only be performed in monitored hospital settings and terminated immediately if ventricular arrhythmias, marked QRS widening, or type 1 pattern develops 1, 3

Universal Lifestyle Modifications (Class I Recommendation)

Every patient with Brugada syndrome must adhere to the following lifestyle changes regardless of symptom status: 1, 2

• Avoid all drugs that induce ST-segment elevation - consult www.brugadadrugs.org for a comprehensive updated list of contraindicated medications including sodium channel blockers (flecainide, ajmaline, procainamide), certain psychotropics (tricyclic antidepressants, some antipsychotics), specific anesthetic agents, and cocaine 1, 3, 4
• Treat any fever immediately and aggressively with antipyretics - fever is a critical trigger accounting for 27% of life-threatening arrhythmic events and can precipitate cardiac arrest 1, 3, 2
• Avoid excessive alcohol intake and large meals - both are known triggers for ventricular fibrillation 1, 3, 2

Common pitfall: One in three patients with Brugada syndrome continues to receive contraindicated medications even after diagnosis, and the diagnosis itself does not change prescription patterns 4. Vigilant medication review at every clinical encounter is essential.

Risk Stratification and ICD Indications

Class I Recommendations (ICD Mandatory)

ICD implantation is recommended for: 1, 2

• Survivors of aborted cardiac arrest - annual event rate 13.5% 1, 2
• Documented spontaneous sustained ventricular tachycardia 1

Class IIa Recommendations (ICD Should Be Considered)

ICD implantation should be considered for patients with spontaneous type 1 ECG pattern AND history of syncope presumed arrhythmic - annual event rate 3.2% 1, 2, 5

Class IIb Recommendations (ICD May Be Considered)

ICD implantation may be considered for patients who develop ventricular fibrillation during programmed ventricular stimulation with two or three extrastimuli at two sites 1

• The prognostic value of electrophysiological study remains debated, as most clinical studies have not confirmed positive or negative predictive value for cardiac events 1
• Asymptomatic patients have an annual event rate of only 1%, but events are not negligible over long-term follow-up (95.4% event-free survival at 10-15 years) 1, 6

Important caveat: 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 1, 2

Pharmacological Therapy

Quinidine (Class IIa Recommendation)

Quinidine should be considered in the following scenarios: 1, 2

• Patients who qualify for ICD but have contraindications or refuse device implantation 1, 2

• Treatment of electrical storms (quinidine or isoproterenol) 1

• Patients requiring treatment for supraventricular arrhythmias 1

• Quinidine reduces ventricular fibrillation inducibility during programmed ventricular stimulation and suppresses the Ito current 1, 2, 7

• One series showed no deaths during mean follow-up over 9 years with quinidine, though 38% experienced adverse effects 1

• Avoid rifampicin as it decreases quinidine plasma concentrations and causes therapeutic failures 3

Catheter Ablation (Class IIb Recommendation)

Catheter ablation of abnormal epicardial late activation areas in the right ventricular outflow tract may be considered for patients with electrical storms or repeated appropriate ICD shocks 1, 5

• Small series show that spontaneous type 1 Brugada pattern may be eliminated in >75% of patients, with marked reduction in VT/VF recurrences 1
• Experience and follow-up remain limited; ICD is still recommended for patients with prior syncope or sudden cardiac arrest even after ablation 1

Genetic Testing (Class IIb Recommendation)

Genetic testing may be considered but does not influence risk stratification or treatment decisions 1

• Yield is approximately 20-30% in phenotype-positive patients, with SCN5A variants accounting for most genotype-positive cases 1
• 2-10% of healthy individuals carry rare SCN5A variants 1
• Genotype status is not correlated with risk of adverse events 1
• Identification of pathogenic mutations helps facilitate family screening and carrier recognition 1, 5

Special Considerations

Drug-induced Brugada pattern (e.g., from procainamide, tricyclic antidepressants like dosulepin) may not require ICD if the patient is low-risk by Shanghai criteria, has no inducible dysrhythmia on electrophysiology study, no family history of sudden death, and no history of syncope 8, 9

• All patients with drug-induced patterns should be referred to cardiology for further evaluation and counseled to avoid Brugada-inducing medications 8
• Discontinuation of the offending agent may resolve the ECG pattern within 24 hours 8, 9