What is the primary management approach for patients diagnosed with Brugada syndrome?

Management of Brugada Syndrome

The primary management approach for Brugada syndrome is risk-stratified: implantable cardioverter-defibrillator (ICD) placement for high-risk patients (those with cardiac arrest, sustained ventricular arrhythmias, or syncope with spontaneous type 1 ECG pattern), observation alone for asymptomatic patients with only drug-induced patterns, and universal lifestyle modifications for all patients regardless of risk category. 1

Risk Stratification Framework

High-Risk Patients Requiring ICD

ICD implantation is the only proven therapy to prevent sudden cardiac death in Brugada syndrome and is mandatory for specific high-risk groups. 1, 2

• Cardiac arrest survivors or documented sustained ventricular arrhythmias: ICD is a Class I recommendation if meaningful survival >1 year is expected 1
• Spontaneous type 1 ECG pattern with recent syncope presumed due to ventricular arrhythmias: ICD should be considered (Class IIa recommendation) 1
• Annual arrhythmic event rates vary dramatically: 13.5% in cardiac arrest survivors, 3.2% in patients with syncope, and only 1% in asymptomatic patients 1, 2

Low-Risk Patients Managed with Observation

Asymptomatic patients with only drug-induced (not spontaneous) type 1 Brugada pattern should be observed without ICD therapy. 1

• Spontaneous type 1 pattern carries significantly worse prognosis than drug-induced pattern 2
• In pediatric populations, spontaneous type 1 ECG pattern and symptoms at diagnosis are the strongest predictors of life-threatening arrhythmias 3

Intermediate-Risk Patients: Electrophysiology Study Consideration

• Electrophysiology study with programmed ventricular stimulation may be considered for further risk stratification in asymptomatic patients with spontaneous type 1 pattern (Class IIb recommendation) 1
• However, the prognostic value of inducible ventricular fibrillation remains controversial, with research showing that asymptomatic patients with inducible VF did not experience arrhythmic events during follow-up 4

Universal Lifestyle Modifications

All patients with Brugada syndrome, regardless of risk category, require specific lifestyle modifications to prevent arrhythmic triggers. 1

• Avoid drugs that induce ST-segment elevation (comprehensive list at www.brugadadrugs.org), including psychotropic medications, certain anesthetics, and cocaine 1
• Aggressive fever management with antipyretics is critical, as fever can acutely precipitate cardiac arrest 1, 2
• Avoid excessive alcohol intake and large meals, which can unmask type 1 ECG pattern and predispose to ventricular fibrillation 1

Management of Recurrent Arrhythmias or ICD Intolerance

Pharmacologic Therapy

Quinidine is the preferred antiarrhythmic medication for Brugada syndrome patients experiencing recurrent ICD shocks or those who cannot receive an ICD. 1, 2, 5

• Quinidine reduces ventricular fibrillation inducibility and is recommended for patients with recurrent ICD shocks (Class I) 1
• Also recommended for patients who qualify for ICD but have contraindications or refuse device implantation 1
• Quinidine or isoproterenol should be considered for treating electrical storms 1

Catheter Ablation

Catheter ablation is recommended as an alternative or adjunct therapy for patients with recurrent ICD shocks or electrical storms. 1

• Epicardial ablation over the anterior right ventricular outflow tract may prevent electrical storms 2
• May be considered in patients with history of electrical storms or repeated appropriate ICD shocks (Class IIb) 1

Diagnostic Confirmation

For suspected Brugada syndrome without spontaneous type 1 pattern, pharmacological challenge with sodium channel blockers (ajmaline, flecainide, procainamide, or pilsicainide) is useful for diagnosis (Class IIa recommendation). 1, 2

• High precordial lead placement (second and third intercostal spaces) improves detection 1
• Testing should be terminated if ventricular arrhythmias, marked QRS widening, or type 1 pattern develops 1

Genetic Testing and Family Screening

Genetic counseling and testing may be useful to facilitate cascade screening of relatives (Class IIb recommendation), though results do not currently influence prognosis or treatment decisions. 1

• SCN5A mutations account for only a small percentage of genetically confirmed cases 1
• In pediatric studies, all patients with life-threatening arrhythmias were genotype-positive, while SCN5A-negative patients remained asymptomatic 3

Critical Pitfalls and Caveats

ICD Complications Are Substantial

The complication rate with ICD therapy in Brugada syndrome is notably high, requiring careful patient selection. 4, 6

• Inappropriate shocks occur in 20-27% of patients, 2.5 times more frequently than appropriate shocks 4, 6
• Common causes include lead failure/dislodgment, T-wave oversensing, sinus tachycardia, and supraventricular tachycardia 4, 6, 7
• Overall complication rate reaches 28-32%, with annual device-related complication rate of 8.9% 4, 6
• Psychiatric assistance required in 13.5% due to ICD-related complications 4

Avoid Contraindicated Medications

Class IC antiarrhythmic drugs (propafenone, flecainide) are absolutely contraindicated in Brugada syndrome, as they can unmask or exacerbate the Brugada ECG pattern and trigger ventricular arrhythmias. 5

Gender and Age Considerations

• Clinical manifestations are eight-fold more frequent in men than women, with mean age of ventricular fibrillation occurrence at 41±15 years 1
• In pediatric populations, management should be age-specific with aggressive use of antipyretics and quinidine, with risk-specific consideration for ICD 3