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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