Brugada Syndrome (BrS) - NYSORA

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Brugada Syndrome (BrS)

Learning objectives

  • Recognize sings & symptoms of BrS
  • Diagnose BrS
  • Manage patients with BrS

Definition & mechanisms

  • Brugada syndrome (BrS) is an abnormality of cardiac ion channels that increases the risk of ventricular fibrillation and sudden cardiac death
  • Linked to 19 genetic mutations that encode for sodium, calcium, or potassium channels and result in either increase or decrease in their activity
  • In up to 80% of patients, no causative genetic mutation can be found
  • Thought to be responsible for up to 40% of sudden cardiac death cases in a structurally normal heart

Signs & symptoms

  • Palpitations
  • Chest discomfort
  • Syncope and nocturnal agonal respiration
  • Monomorphic ventricular tachycardia is rare but is more often seen in infants and children
  • Events typically occur during sleep with increased vagal tone, with fever, or can be precipitated by drugs, alcohol, and electrolyte disorders
  • Between 20 and 30% of patients: supraventricular tachycardias (atrial flutter, atrioventricular nodal re-entry, Wolff-Parkinson-White syndrome), atrial fibrillation is seen most frequently
  • In critical care, the most common presentation will be a patient with aborted sudden cardiac death
  • Many patients remain asymptomatic

Diagnosis

Diagnosis is based on fulfilling the BrS ECG morphological criteria:

  • Type 1: A cove shaped (with T-wave inversion) ST-segment elevation 2 mm in V1 and/or V2 when placed in a standard or superior position, either spontaneously or after Na-channel blocking agent administration (e.g., ajmaline/flecainide)
  • Additional ECG morphologies: 
    • Type 2: Saddleback-shaped (with positive T-wave) ST-segment 1 mm in V1 and/or V2
    • Type 3: Saddleback or cove-shaped ST-segment elevation <1 mm in V1 and/or V2
    • Neither are diagnostic
  • The ECG morphology can change with time and an individual with true BrS can manifest all three different morphologies at different times
  • Many conditions can reproduce a type 1 Brugada ECG, differential diagnosis:

Management

brugada syndrome, ecg, syncopal, agonal respiratkon, tachycardia, fibrillation, qrs, t-wave, repolarization, sudden cardiac death, antiarhythmic drugs, lithium, tricyclic antidepressants, amiodarone, verapamil, alpha agonist, beta blocker, tetracyclic, antiepileptics, carbamazepine, phenytoin, lamotrigine, electrolytes, magnesium, icd, defibrillation, isoprenaline, quinidine, propofol, thiopental, ketamine, etemidate, opioids, lidocaine, bupivacaine, pyrexia, icu, antipyretics

Suggested reading

  • Levy D, Bigham C, Tomlinson D. Anaesthesia for patients with hereditary arrhythmias part I: Brugada syndrome. BJA Educ. 2018;18(6):159-165.

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