56  Brugada Syndrome

• Diagnostic criteria (Source)
  • ST elevation of 2mm or more with type 1 (“coved”) morphology seen in 1 or more of leads V₁-V₃
  • occurs spontaneously or provoked by Na-channel blockers
  • other causes of STE have been ruled-out (RV or LAD ischemia, acute PE)
• Pathophysiology
  • Related to loss of function of Na-channels
  • Genetic link with SCN5A gene mutations, which are found in 30%
  • Autosomal dominant inheritance pattern but incomplete penetrance & variable expression
• Presentation
  • Syncope, nocturnal agonal breathing from polymorphic VT (PMVT)/VF or sudden cardiac death
  • PMVT/VF are assoc w/ bradycardia or vagal events → commonly occur when sleeping (old name: Sudden Unexplained Nocturnal Death Syndrome)
  • Fever also a trigger
• Management
  • Antipyretics
  • If symptomatic: ICD placement
  • Patients who decline ICD or have recurrent ICD shocks → quinidine, amio or consider catheter ablation

Appearance of ECG in Brugada syndrome.