Brugada Syndrome

Background

• Brugada syndrome is an inherited arrhythmia caused by genetic mutations that disrupt the function of proteins regulating cardiac ion channels that is associated with risk of ventricular fibrillation and subsequent sudden cardiac death.
• Symptoms associated with Brugada syndrome are more common in men than women and the incidence is higher in patients of Asian descent, especially Southeast Asian descent.

Evaluation

• Use electrocardiography (ECG) for diagnosis of Brugada type I pattern (coved ST-segment elevation ≥ 2 mm) on ≥ 1 right precordial leads (V1 and V2) positioned in second, third, or fourth intercostal space
• Consider ECG pharmacologic challenge with sodium channel blocker in patients with suspected Brugada syndrome without spontaneous Brugada type I ECG pattern (Weak recommendation).

Management

• General management:
  • Observation without therapy is recommended for asymptomatic patients with only drug-inducible Brugada type I ECG pattern (no spontaneous Brugada type I ECG pattern) (Strong recommendation).
  • Avoid medications that may induce or aggravate ST-segment elevation in the right precordial leads (for examples, see Brugada Drugs website) (Strong recommendation).
  • Treat fever immediately with antipyretic medications (Strong recommendation).
• Implantable cardioverter defibrillator (ICD):
  • Use an ICD to treat survivors of cardiac arrest and patients with spontaneous sustained ventricular tachycardia with or without syncope (Strong recommendation).
  • Use an ICD to treat patients with spontaneous Brugada type I ECG pattern if cardiac arrest, sustained ventricular arrhythmias, or recent history of syncope presumed to be due to ventricular arrhythmia and meaningful survival > 1 year expected (Strong recommendation).
  • Consider an ICD for patients with spontaneous type 1 ST-segment elevation on electrocardiogram (ECG) and a history of syncope likely associated with ventricular arrhythmias (Weak recommendation).
  • Consider an ICD for patients with Brugada type I ECG pattern who develop ventricular fibrillation during programmed electrical stimulation (Weak recommendation).
  • Do not use an ICD for asymptomatic patients with drug-induced type 1 ST-segment elevation on ECG with a family history of sudden cardiac death (Strong recommendation).
• Consider treatment with catheter ablation or quinidine to patients with spontaneous Brugada type I ECG pattern and symptomatic ventricular arrhythmias who are not candidates for or refuse ICD implantation (Weak recommendation)
• Consider catheter ablation for patients with a history of:
  • arrhythmic storms, defined as ≥ 2 episodes of ventricular tachycardia/ventricular fibrillation in 24 hours (Weak recommendation)
  • repeated appropriate ICD shocks (Weak recommendation)
• Consider quinidine in patients:
  • with a history of arrhythmic storms, defined as ≥ 2 episodes of ventricular tachycardia/ventricular fibrillation in 24 hours (Weak recommendation)
  • who qualify for an ICD but either refuse or have contraindications to its use (Weak recommendation)
  • with a history of supraventricular arrhythmias that require treatment (Weak recommendation)
  • that are asymptomatic with spontaneous type 1 ST-segment elevation on ECG (Weak recommendation)
• Isoproterenol infusion can be helpful for suppressing arrhythmic storms (Weak recommendation).