What is the management approach for a patient with suspected Brugada syndrome, particularly in terms of diagnostic testing, medication, and prevention of sudden cardiac death?

Management of Brugada Syndrome

An implantable cardioverter-defibrillator (ICD) is the definitive treatment for preventing sudden cardiac death in Brugada syndrome patients with cardiac arrest, sustained ventricular arrhythmias, or spontaneous type 1 ECG pattern with syncope presumed due to ventricular arrhythmia. 1

Diagnostic Approach

ECG Criteria for Diagnosis

• Brugada syndrome is diagnosed when ST-segment elevation with type 1 morphology ≥2 mm occurs in one or more leads among right precordial leads V1 and/or V2, positioned in the second, third, or fourth intercostal space, either spontaneously or after provocative drug testing. 1
• Sodium channel blockers (ajmaline, flecainide, procainamide, or pilsicainide) can unmask the diagnostic pattern in patients with suspected Brugada syndrome who lack spontaneous type 1 ECG changes 1
• Patients with spontaneous type 1 pattern have significantly worse prognosis than those requiring pharmacological provocation 1

Risk Stratification by Clinical Presentation

The annual arrhythmic event rates stratify patients clearly 1, 2:

• Cardiac arrest survivors: 13.5% per year
• Syncope patients: 3.2% per year
• Asymptomatic patients: 1% per year

Patients with spontaneous type 1 ECG and syncope have 6-fold higher cardiac arrest risk compared to those without syncope 1

ICD Indications (Class I Recommendations)

ICD implantation is recommended (Class I) for: 1

• Survivors of aborted cardiac arrest
• Documented spontaneous sustained ventricular tachycardia
• Spontaneous type 1 ECG pattern with history of syncope presumed due to ventricular arrhythmia

ICD should be considered (Class IIa) for: 1

• Patients with spontaneous diagnostic type 1 ECG and history of syncope

Asymptomatic Patients

Observation without therapy is recommended for asymptomatic patients with only inducible type 1 Brugada pattern. 1 The role of electrophysiological testing remains controversial—it may be considered for risk stratification in asymptomatic patients with spontaneous type 1 pattern, but has limited predictive accuracy 1

Medical Management

Mandatory Lifestyle Modifications (Class I)

All diagnosed patients must implement these measures 1, 2:

• Avoid drugs that induce ST-segment elevation (comprehensive list at www.brugadadrugs.org, including sodium channel blockers, certain psychotropic medications, and anesthetic agents) 1, 2
• Avoid excessive alcohol intake and large meals 1
• Prompt treatment of fever with antipyretic drugs—fever is a critical trigger for ventricular fibrillation and requires aggressive early treatment 1, 2

Quinidine Therapy

Quinidine should be considered (Class IIa) for: 1

• Patients who qualify for ICD but have contraindications or refuse device implantation
• Treatment of supraventricular arrhythmias in Brugada patients
• Patients with spontaneous type 1 pattern and symptomatic ventricular arrhythmias who are not ICD candidates or decline ICD 1

Management of Electrical Storms

For patients experiencing recurrent ICD shocks or electrical storms: 1

• Quinidine or isoproterenol should be considered for acute management 1
• Intensification of therapy with quinidine or catheter ablation is recommended 1
• Catheter ablation may be considered for patients with history of electrical storms or repeated appropriate ICD shocks 1

Genetic Testing and Family Screening

• Genetic counseling and testing may be useful to facilitate cascade screening of relatives (Class IIb), though results do not currently influence prognosis or treatment 1, 2
• SCN5A mutations account for 20-30% of cases, but negative genetic testing does not exclude diagnosis—Brugada syndrome remains primarily a clinical and electrocardiographic diagnosis 1, 2
• Family screening with ECG is recommended for all first-degree relatives 2

Common Pitfalls to Avoid

• Do not rely on family history for individual risk stratification—family history does not predict cardiac events among family members 1
• Do not use programmed ventricular stimulation as sole determinant for ICD—its predictive value remains debated, with conflicting evidence on positive and negative predictive values 1
• Do not overlook fever as a trigger—it is a major precipitant of ventricular fibrillation and requires immediate aggressive antipyretic treatment 1, 2
• Recognize that the ECG pattern can be transient—normalization occurs in many patients, requiring provocative testing for diagnosis 1, 3