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Brugada Syndrome

Brugada Syndrome is an abnormal ECG (Right Bundle Branch Block Pattern with coved ST elevation over the right precordial leads of V1-V3), which leads to ventricular fibrillation (VF) and sudden cardiac death (SCD) in patients with structurally normal hearts. It has been recognized as a clinical entity since 1992. Why should all ED physicians know about this entity? Although a rare syndrome, it is often mistaken as a STEMI and more importantly the clinical spectrum can be asymptomatic to SCD.

Who gets Brugada Syndrome?

  • Males  > Females in a 8 – 10: 1 ratio
  • Ages 20 – 40 years (There are case reports of age 2 days all the way up to 84 years)
  • Asian > US populations
  • Typically occurs at night, when there is a predominance of vagal activity

 How common is Brugada Syndrome?

  • Worldwide 4 – 12% of all sudden deaths
  • Type 1 Brugada occurs in 12/10,000 people [1]
  • Type 2 and 3 Brugada occurs in 58/10,000 people [1]
  • Prevalance of Brugada Pattern ECG: Asia (0.36%), Europe (0.25%), and in the USA (0.03%) [2]
  • ECG pattern can wax and wane, making the true incidence underestimated

What are the types of Brugada Syndrome?

  • Type 1, Type 2, and Type 3 from left to right, seen in leads V1, V2, or V3.

Brugada Types 1 Brugada Types 2 Brugada Types 3

Where is the most likely arrhythmogenic substrate of Brugada Syndrome?

  • Right Ventricular Outflow Tract (RVOT)
  • Only cardiac structure lying underneath 2nd and 3rd intercostal spaces
  • Brugada pattern may be absent in typical 4th intercostal space of leads V1 – V3
  • Some drugs may induce VF/VT and a comprehensive list can be found at http://www.brugadadrugs.org

What is the best way to risk stratify patients with Brugada Syndrome?

  • Symptomatic patients with recurrent syncope, agonal respirations at night during sleep, or unknown seizures are at the highest risk of dying
  • Asymptomatic patients have an annual cardiac event rate of 0.25%, therefore there is little value in a risk stratification strategy to identify high risk patients

What are the treatment options for Brugada Syndrome?

  • Quinidine is the only medication that has shown benefit in prevention of VF and reduction of AICD shocks (Only 67% of patients can tolerate drug due to side effects)
  • Implantable Cardiac Defibrillator (ICD): Class 1 Indication in symptomatic patients (past history of VT/VF or syncope)
  • Defibrillator Versus B-Blocker in Unexplained Death in Thailand (DEBUT) Trial: Showed 0% death rate after ICD versus 18% in Beta Blocker group [3]
  • Leadless ICDs: 98% termination rate of VF/VT, but less pocket infection and lead revisions [4]
  • Catheter Ablation: Performed in 14 patients with no recurrent VF/VT with a median 32 month follow up [5]

 Risk Stratification

Image from [4]

Take home point

Brugada Syndrome is an uncommon entity, that can mimic STEMI, has a high rate of sudden cardiac death, and currently ICD placement is the best treatment strategy.

References

  1. Y. Miyasaka, H. Tsuji, K. Yamada, S. Tokunaga, D. Saito, Y. Imuro, N. Matsumoto, and T. Iwasaka, "Prevalence and mortality of the Brugada-type electrocardiogram in one city in Japan.", Journal of the American College of Cardiology, 2001. http://www.ncbi.nlm.nih.gov/pubmed/11527631
  2. Y. Mizusawa, and A.A.M. Wilde, "Brugada syndrome.", Circulation. Arrhythmia and electrophysiology, 2012. http://www.ncbi.nlm.nih.gov/pubmed/22715240
  3. K. Nademanee, G. Veerakul, M. Mower, K. Likittanasombat, R. Krittayapong, K. Bhuripanyo, S. Sitthisook, L. Chaothawee, M.Y. Lai, and S.P. Azen, "Defibrillator Versus beta-Blockers for Unexplained Death in Thailand (DEBUT): a randomized clinical trial.", Circulation, 2003. http://www.ncbi.nlm.nih.gov/pubmed/12695290
  4. J. Jellins, M. Milanovic, D. Taitz, S.H. Wan, and P.W. Yam, "Brugada syndrome.", Hong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine, 2013. http://www.ncbi.nlm.nih.gov/pubmed/23535677
  5. R. Zügner, R. Tranberg, P. Herberts, B. Romanus, and J. Kärrholm, "Stable fixation but unpredictable bone remodelling around the Lord stem: minimum 23-year follow-up of 66 total hip arthroplasties.", The Journal of arthroplasty, 2012. http://www.ncbi.nlm.nih.gov/pubmed/23142437
Salim Rezaie, MD

Salim Rezaie, MD

ALiEM Associate Editor
Clinical Assistant Professor of EM and IM
University of Texas Health Science Center at San Antonio
Founder, Editor, Author of R.E.B.E.L. EM and REBEL Reviews
Salim Rezaie, MD