What is the primary treatment for a patient with Brugada syndrome, particularly those with a history of cardiac arrest, syncope, or a family history of sudden cardiac death?

Treatment for Brugada Syndrome

An implantable cardioverter-defibrillator (ICD) is the only proven treatment to prevent sudden cardiac death in Brugada syndrome and is mandatory for high-risk patients, including cardiac arrest survivors and those with spontaneous type 1 ECG pattern plus syncope. 1

Risk-Based Treatment Algorithm

Highest Risk: Cardiac Arrest Survivors or Documented Sustained VT

• ICD implantation is Class I recommendation (annual event rate 13.5%) 1
• These patients had appropriate ICD therapy in 8% of cases during follow-up, with all appropriate shocks occurring exclusively in this group 2, 3

High Risk: Spontaneous Type 1 ECG + Syncope

• ICD implantation should be strongly considered (annual event rate 3.2%) 1
• The 2017 ACC/AHA/HRS guidelines give this a Class I recommendation when meaningful survival >1 year is expected 1
• Spontaneous type 1 pattern carries significantly worse prognosis than drug-induced pattern 4

Intermediate Risk: Asymptomatic with Spontaneous Type 1 ECG

• Observation without therapy is recommended (annual event rate 1%) 1
• Electrophysiological study with programmed ventricular stimulation may be considered for further risk stratification, though its predictive value remains debated 1
• In research cohorts, asymptomatic patients without prior cardiac arrest had zero appropriate ICD therapies during 39-month follow-up 2

Asymptomatic with Only Drug-Induced Type 1 ECG

• Observation without therapy is the standard approach 1

Universal Lifestyle Modifications (All Patients)

Regardless of risk category, all Brugada patients must implement these measures:

• 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 anesthetics 1, 4, 5
• Treat any fever immediately and aggressively with antipyretics, as fever precipitates 27% of life-threatening arrhythmic events and can acutely trigger cardiac arrest 1, 4, 5
• Avoid excessive alcohol intake and large meals, both established VF triggers 1, 4, 5

Alternative Therapies for Specific Scenarios

Quinidine Therapy

Quinidine is recommended (Class I) for patients experiencing recurrent ICD shocks for polymorphic VT 1

Additional quinidine indications (Class IIa):

• Patients who qualify for ICD but have contraindications or refuse the device 1, 4
• Treatment of electrical storms (in combination with isoproterenol) 1
• Patients requiring treatment for supraventricular arrhythmias 1

Quinidine reduces VF inducibility during programmed ventricular stimulation, though data confirming reduction in sudden cardiac death risk are lacking 1

Catheter Ablation

Epicardial catheter ablation over the anterior right ventricular outflow tract is recommended (Class I) for patients with recurrent ICD shocks 1

• Results in non-inducibility of ventricular arrhythmias in 91% and freedom from arrhythmias in 87% during 30-month follow-up 6
• May be considered (Class IIb) for electrical storms or repeated appropriate ICD shocks 1

Critical Caveats and Complications

ICD-Related Complications

The complication rate is substantial in this young, otherwise healthy population:

• Inappropriate shocks occur in 20-27% of patients (2.5 times more frequent than appropriate shocks), with annual inappropriate shock rate of 8.9% 3, 7
• Common causes: lead failure/dislodgment, T-wave oversensing, sinus tachycardia, supraventricular tachycardia, and new-onset atrial fibrillation 2, 3, 7
• Overall complication rate reaches 28-32%, including lead problems requiring reintervention (18.6%), device malfunction, and psychological distress requiring psychiatric assistance (13.5%) 2, 3
• New-onset atrial fibrillation and age <50 years are independent predictors of inappropriate shocks 7

Diagnostic Considerations

• Pharmacological challenge with sodium channel blocker (ajmaline, flecainide, or procainamide) is useful for diagnosis when spontaneous type 1 pattern is absent 1
• Genetic counseling and testing may be useful to facilitate cascade screening of first-degree relatives, though SCN5A mutations account for only 20-30% of cases and do not influence treatment decisions 1, 4

Epidemiologic Context

• Disease predominantly affects males (8-10 times more frequent than females) with mean VF age of 41±15 years 1, 5
• Arrhythmias typically occur during rest or sleep 1, 5
• Prevalence is higher in Southeast Asia (1 in 1,000 to 1 in 10,000) 1, 5