Brugada Syndrome: An ECG Pattern You Need to Know

Brugada Syndrome: An ECG Pattern You Need to Know

2016-11-11T19:02:20+00:00

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.

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 2
  • Catheter Ablation: Performed in 14 patients with no recurrent VF/VT with a median 32 month follow up 4
Risk Stratification

Image from 2

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.

1.
Miyasaka Y, Tsuji H, Yamada K, et al. Prevalence and mortality of the Brugada-type electrocardiogram in one city in Japan. J Am Coll Cardiol. 2001;38(3):771-774. [PubMed]
2.
Mizusawa Y, Wilde A. Brugada syndrome. Circ Arrhythm Electrophysiol. 2012;5(3):606-616. [PubMed]
3.
Nademanee K, Veerakul G, Mower M, et al. Defibrillator Versus beta-Blockers for Unexplained Death in Thailand (DEBUT): a randomized clinical trial. Circulation. 2003;107(17):2221-2226. [PubMed]
4.
Zügner R, Tranberg R, Herberts P, Romanus B, Kärrholm J. Stable fixation but unpredictable bone remodelling around the Lord stem: minimum 23-year follow-up of 66 total hip arthroplasties. J Arthroplasty. 2013;28(4):644-649. [PubMed]
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
  • mark olgun

    Is it true that the classic Brugada pattern disappears within minutes after a Brugada related syncopal episode..?? hence, the importance of a quick 12 lead acquisition following a syncopal episode..

    • Salim R. Rezaie

      Many studies describe this phenomenon of transient ECG normalization, one series from 1997 that I know of that describes this phenomenon http://www.ncbi.nlm.nih.gov/pubmed/9083883?dopt=Abstract . 14/47 (29.8%) patients normalized after syncopal event. Other studies and case reports describe normalization of ECG as well, which is why it is thought that the syndrome is actually under diagnosed because it can be transient and missed.

      There is no study that documents time from syncope to ECG looking at Brugada Syndrome that I am aware of, but primary dx is made by ECG and if suspected, serial ECGs can be helpful. Hope this was helpful and thank you for reading the post and your comment.

      Salim

      • Michelle

        Thanks for the quick response, Salim. It’s all the more reason to take a close look at all prehospital ECG’s to see if we can capture this elusive pattern.

  • Singapore Joe

    Hi, during recent hospitalization the Brugada pattern appeared on an ECG which was taken during the time I had high fever presumably caused by virus. I have never had any syncopal events, or other Brugada Sydnrome symptoms and the ECG became normal after the fever subsided. I have had ECGs in the past which have been normal. I have some history of anxiety and cardiac arrythmias but these have always been diagnosed as benign. I had one isolated episode of confirmed Atrial Fibrillation around 6 years ago which has never re-occurred. I have also had various cases of post exercise tachycardia in the past which doctors have found difficult to explain and believe may be caused by anxiety and it was suggested I take beta blockers before exercise. After the latest event occurred I had various tests – angiogram and echo tests show no blockages and normal heart structure and functioning. My question is what is the current recommendation for someone like me? I am told that due to absence of any other symptoms EPS is not recommended for someone like me. Any thoughts or references would be appreciated.

    • Singapore Joe

      Sorry, I should have mentioned that I am 55 years old.

      • Michelle

        Hello, thanks for sharing your experience. We are currently not providing direct patient advice. Please contact your physician.

  • Charlie Diaz

    I was currently diagnosed with brugada syndrome type 1. I had finished my shift (I’m a Paramedic) and had palpitations. I had these sudden palpitations (rarely) lasting about 30 seconds. I gave myself an EKG before but never found anything. I was working long hours and out of no where i felt my pulse become irregular. I preformed an EKG on myself and found myself in Sinus Arrhythmia with Unifocal PVC’s. The rate of the PVC’s was about once every 6 beats. At first It didn’t cause me much concern. What made me go to the hospital was I started feeling my pulse irregular and had an EKG present It probably would have shown bigeminy unifocal PVC’s. Thankfully, Colombia Presbyterian Hospital in New York City diagnosed me and a day later had an ICD placed. I haven’t felt any irregular beats since then. I only pray there are other discoveries that can help people with Brugada Syndrome. I just wanted to share my story and hope it helps people with an unsuspecting symptoms.

  • Jessica

    Just discovered this forum and Brugada Syndrome. My father died suddenly in his sleep after a 10 year history of arrhythmia problems, despite the fact that he had an implanted defibrillator. My uncle, my father’s brother, also died in his sleep after an unexplained cardiac event and no prior arrythmia history (they assume–his heart looked normal and healthy at autopsy). Their sister also had a sudden cardiac event in her sleep, but her husband noticed and revived her. She was then diagnosed with arrythmias and put on medication. Each of them was in their upper 50’s and 60’s when their problems manifested. I am now experiencing fluttering sensations and palpitations in my mid-40’s. Could be stress or indigestion, but with my family history, who knows? EKG at Drs office was normal; was referred to cardiologist for further exploration. Doing research now on family history and arrythmias, but not a lot out there. Would a cardiologist generally be familiar with Brugada syndrome since it is fairly rare? We are not of Asian descent, just European and Native American ancestors; so it seems not to match our history due to age of onset, and the fact that I haven’t suddenly died in my sleep. Are there other genetic arrythmia disorders that fit our family history?