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:
  • Early repolarisation
  • Athlete’s heart
  • Acute coronary events
  • Pulmonary embolism
  • Electrolyte disturbance
  • Pericarditis
  • Myocarditis
  • Dissecting aortic aneurysm
  • Arrhythmogenic right ventricular cardiomyopathy

Management

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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