Thursday, August 22, 2013

Brugada Syndrome

This 33 y/o male who came c/o palpitation episodes for very long time and more recent near syncopal episodes has Brugada syndrome. This, my friends, is VERY important diagnosis to make in the ED. Many, and by that I mean a lot, of ED docs and even cardiologist don't recognize the Brugada pattern. That is a sad, b/c we are in the business of saving lives and not making this diagnosis is a missed opportunity to save one and bad for business.

First, a little bit of history. Brugada is a relatively new electrocardiographic diagnosis considering that electrocardiography has been in use for almost 100 years, Brugada was discovered in the late 80's. The Brugada brothers (they are 9 super smart cardiologist) collected case series of young patients, mostly men and many of Asian origen, who had sudden cardiac death. Looking back in the available histories, these patients often complained of palpitations, dizzy spells, syncope or near syncope episodes and some had family history of unexplained deaths in young relatives. For those who had EKG's in files the pattern of down-slopping ST segment in V1-V3 with a complete or incomplete RBBB pattern was a constant finding. As technology advanced and genetic testing became more sophisticated, different gene mutations of the potassium channels were identified that predispose patients to fatal arrhythmias mainly polymorphic VT, VT/VF. Often times they self terminate and the only history we get is that of palpitations, syncope or near syncope, but in some cases sudden cardiac death is the presenting symptom of Brugada.

OK, enough of that... now let's see the EKG of this patient I saw few weeks ago. Note the STE in V1-V2. This is not the STE of ischemia, early repolarization or pericarditis, this is a down-slopping STE. This can have some small variations in the shape depending of what mutation is present, but the key point is that you are not supposed to have that STE pattern. Often it is associated with long QT, which is another important genetic mutation on it self with risk of Torsades de Points, and both can be seen in the same patient. The QT in this case in 454, which is in the borderline of prolonged.

Brugada is classified in different types, depending of the morphology and the genetics. But in all types, the down-slopping STE is the key to make the diagnosis. If it looks like a saddle or not is irrelevant, the key here is to identify Brugada. Look at the 3 types below, now close your eyes and make a mental picture for your long term memory. (I try that sometimes and occasionally it works :)

Another key feature of Brugada that you must not forget is about treatment. That is very easy b/c there is only one. The only treatment proven to decrease mortality is an intracardiac defibrillator. Anti-arrhythmics don't work, beta-blockers don't work, calcium blockers don't work, magnesium doesn't work, gummy bears don't work. That's why it is important we make this diagnosis and refer to an electrophysiologist pronto, so these patients can get their ICD and survive.

Now, this is another case I had few years ago. This guy had been seen in different ED's, seen by cardiologist, diagnosed with anxiety and let loose.

Again, note the down-slopping STE in V1-2. On the side, this guy also have a PVC, which may suggest some ventricular irritability as well. When I told this guy about the dx and tx, he asked me - Why no one told me about this? I have been seeing doctors for years! - He went to Germany to have his ICD and now has a normal life.

Key points

1.- Remember Brugada in every patient with history of palpitations, syncope, near syncope or family h/o unexplained deaths.
2.- Recognize the down-slopping STE in V1-3 with complete or incomplete RBBB pattern. That is the landmark of Brugada.
3.- The only treatment is an ICD. These patients need referral to an electrophysiologist, pronto!
4.- Gummy bears are good but not for Brugada.

Now, if you are really into EKG's, you may want to read more about this topic. Here is a complete review on Brugada from the cardiology literature. Maybe a bit too much, but interesting reading nevertheless.

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