Brugada Syndrome

Inheritable condition which accounts for part of a group of patients with idiopathic ventricular fibrillation who have no evidence of causative structural cardiac disease

Aetiology

  • 12 associated genes - cardiac sodium and calcium channels
  • Autosomal dominant, 8x more likely in males

Pathophysiology

VF triggers

  • Usually rest or sleep
  • Fever
  • Excessive alcohol, large meals
  • Drug triggers include β-blockers, psychotropics, analgesics and anesthetics

Clinical presentation

  • Risk of polymorphic VT, and VF
  • AF common

Investigations

ECG

  • ST elevation and RBBB in V1-V3
  • ECG findings may be intermittent and change over time
  • Diagnostic changes may only be seen with provocation testing
notion image

Management

  • Extend genetic testing to family
  • ICD
  • Avoid triggers