What is the incidence and prevalence of Brugada syndrome?

Incidence and Prevalence of Brugada Syndrome

The prevalence of Brugada syndrome ranges from 1 in 1000 to 1 in 10,000 individuals, with significantly higher prevalence in Southeast Asia than in Western countries. 1

Geographical Distribution

Brugada syndrome shows marked geographical variation in its prevalence:

• Southeast Asia: Highest prevalence at approximately 1.8 per 1,000 people (0.18%) 2
• Western countries: Lower prevalence, typically between 0.05-0.1% 3
• France (Lorraine region): Estimated prevalence of 0.3% in a screening study 4

The worldwide pooled prevalence has been estimated at 0.5 per 1,000 individuals (0.05%) 2. This geographical variation reflects genetic differences between populations, with Brugada syndrome being approximately:

• 9 times more common in Asians than in Caucasians
• 36 times more common in Asians than in Hispanics 2

Demographic Patterns

Brugada syndrome demonstrates clear demographic patterns:

• Sex distribution: Males are affected approximately 8 times more frequently than females 1
• Age of manifestation: Ventricular fibrillation typically occurs at a mean age of 41 ± 15 years 1
• Inheritance pattern: Autosomal dominant with variable penetrance 1

Incidence of Arrhythmic Events

The annual incidence of arrhythmic events (sustained ventricular tachycardia, ventricular fibrillation, appropriate ICD therapy, or sudden death) varies significantly based on risk profile:

• Patients with prior cardiac arrest: 13.5% per year
• Patients with syncope: 3.2% per year
• Asymptomatic patients: 1% per year 1

Genetic Aspects

At least 12 genes have been associated with Brugada syndrome, but only two (SCN5A and CACN1Ac) individually account for more than 5% of genetically confirmed cases 1. Recent evidence suggests a complex polygenic inheritance pattern rather than a simple monogenic disorder 5.

Clinical Implications

Understanding the prevalence and incidence of Brugada syndrome is critical for:

1. Risk stratification: Patients with spontaneous type 1 ECG pattern and history of syncope have a 6-fold higher risk of cardiac arrest compared to those without syncope 1

2. Regional healthcare planning: Higher vigilance is needed in Southeast Asian populations where prevalence is significantly higher

3. Preventive measures: Recognition of triggers that can unmask Brugada pattern and precipitate arrhythmias, including:

   • Fever
   • Excessive alcohol intake
   • Large meals 1

The epidemiological data underscores the importance of targeted screening and appropriate management strategies, particularly in high-risk populations and in patients with unexplained syncope or family history of sudden cardiac death.