A 16 year-old male presents to the ED after a syncopal episode. He currently feels well and wants to go home. Below is his EKG, what do you think?





The above presentation (syncopal event) paired with this EKG is concerning for the Brugada syndrome.


  • The Brugada syndrome was first described in 1992 by Drs. Pedro and Josep Brugada in a case report of 8 patient with aborted sudden cardiac death (SCD), though cases of sudden unexplained nocturnal death (SUND) had been described previously in various southeast Asian populations (Juang JM et al, 2004).
  • Some estimate that 4-12% of SCD can be attributed to the syndrome, with a higher prevalence in SE Asian populations (Gourraud JB et al, 2016)
  • Median age of presentation is 41, though has be reported in neonates through the elderly.
  • While many mutations have been described, the Brugada syndrome appears to result from mutations in the Na+ channel, affecting the SCN5A gene (Behere SP et al, 2015)
    • Note: Only 20% of patients with Brugada syndrome have a documented mutation in the SCN5A gene. Studies show a autosomal dominant transmission with incomplete penetrance  (Juang JM et al, 2004).

Clinical Presentation:

  • In the original description of the syndrome, the Brugada brothers noted that all patients presented with syncope, with 5/7 progressing immediately to cardiac arrest and the other 2 progressing to cardiac arrest over the next 2 weeks
    • Registry data indicates that almost 1/3 present with syncope, 6% with SCD, though >60% were asymptomatic at the time of diagnosis (Probst V et al, 2010)
      • NOTE: Registry listed above excluded children, so interpret with caution
  • Sustained Polymorphic Ventricular Tachycardia is the most common rhythm noted to precipitate SCD

EKG Findings and Diagnosis

  • ST elevation in V1-3 with right bundle block morphology is common, with “coved pattern” in V1-3  negative T wave (referred to as “Type 1;” this is the only pathognomonic EKG pattern)
    • In most cases, no reciprocal ST-depression noted (Juang JM et al, 2004).
  • EKGs in Brugada syndrome tend to be variable and at times are even normal
  • In patients with normal EKG, class Ia or Ic antiarrhythmic drugs ( potently blocks Na channel) may unmask the Brugada pattern (Juang JM et al, 2004).
  • Two other patterns have been described, though are considered less significant
  • To be considered diagnostic of Brugada syndrome the EKG findings must be combined with the following clinical findings:
    • Polymorphic ventricular tachycardia
    • syncope
    • ventricular fibrillation
    • Family history of early sudden cardiac death (<45)
  • Workup must also exclude other causes that lead to ST-elevation (e.g. acute pericarditis, arrhythmogenic right ventricular dysplasia (ARVD), Prinzmetal angina, etc).


  • Implantable cardioverter-defibrillator (ICD) is the only strategy to prevent SCD
  • Patients should also be advised to avoid alcohol, certain drugs, and treat fevers aggressively (Refaat MM, 2016).
Resident Reviewer: Vanessa Tommey, MD


  1. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a multicenter report. J Am Coll Cardiol 1992;20:1391–6
  2. Juang JM et al. “Brugada syndrome–an under-recognized electrical disease in patients with sudden cardiac death.” Cardiology. 2004;101(4):157-69
  3. Gourraud JB et al. “The Brugada Syndrome: A Rare Arrhythmia Disorder with Complex Inheritance.” Front Cardiovasc Med. 2016 Apr 25;3:9.
  4. Behere SP et al. “Inherited arrhythmias: The cardiac channelopathies.” Ann Pediatr Cardiol. 2015 Sep-Dec; 8(3): 210–220.
  5. Probst V et al. “Long-Term Prognosis of Patients Diagnosed With Brugada Syndrome.” Circulation. 2010;121(5):635-643.
  6. Alley P. “What is Brugada Syndrome?”