Brugada Syndrome

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Basics

Description

  • Inherited heart disease due to mutations of cardiac Na+ channels without structural abnormalities
  • Very high risk of sudden cardiac death in the form of ventricular fibrillation
  • 2-yr mortality ∼30%
  • Suspected in 40–60% of what was previously known as idiopathic ventricular fibrillation
  • Higher prevalence in men of Southeast Asian descent, but all ages, genders, races can be affected

Etiology

  • Inherited:
    • Autosomal dominant in 50%
    • Variable penetration
  • Cardiac Na+ channel:
    • >70 described mutations
    • Variable penetrance
    • SCN5A mutations account for 20%

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Basics

Description

  • Inherited heart disease due to mutations of cardiac Na+ channels without structural abnormalities
  • Very high risk of sudden cardiac death in the form of ventricular fibrillation
  • 2-yr mortality ∼30%
  • Suspected in 40–60% of what was previously known as idiopathic ventricular fibrillation
  • Higher prevalence in men of Southeast Asian descent, but all ages, genders, races can be affected

Etiology

  • Inherited:
    • Autosomal dominant in 50%
    • Variable penetration
  • Cardiac Na+ channel:
    • >70 described mutations
    • Variable penetrance
    • SCN5A mutations account for 20%

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