There is not just one Brugada, but a family of them!
It all began in 1987 when Professor Brugada was running the electrophysiology laboratory at the University of Maastricht in the Netherlands and a Polish man brought his son to see him. The boy was 3 years old and had a history of repeated episodes of fainting and cardiac arrest; his father had resuscitated him several times. His sister had suffered similar symptoms and had died at the age of 3 in spite of treatment with a pacemaker and amiodarone.
“The electrocardiogram of that boy was never seen before and something you could not find in any publication,” Professor Brugada remembers. “I was very fortunate that the father was able to return to Poland and bring the electrocardiograms of the sister, which turned out to be exactly the same as those of the brother. Remember, it was 1987; the Berlin Wall was still there, so everything that this guy was doing was illegal.”
It took 4 years for the Brugadas to find 2 more patients with similar ECGs – one from the Netherlands and the other from Belgium. They presented the 4 cases as an abstract to an American Heart Association conference, and several doctors who had come across similar cases contacted them. A year later, they published details of 8 patients [in JACC: http://www.sciencedirect.com/science/article/pii/073510979290253J%5D.
Secondary Prevention of death due to VF/VT in settings of:
1. prior VT/VF req’ing resuscitation, or unstable VT with unknown cause
-includes idiopathic vf/vt and congenital long qt
-excludes vt/vf w/in 48hrs of MI
2. spontaneous sustained VT in presence of heart disease (valvular, ischemic, hypertrophic, dilated, infiltrative cardiomyopathy or channelopathies)
For the primary prevention of VT/VF in patients at risk of sudden cardiac death due to vf/vt, who are optimized under medical management, such as:
1. Prior MI (40+ days ago) and LVEF <= 30%
2. Cardiomyopathy, NYHA class 2-3 + LVEF <=35% ( if nonischemic this means 3 months of med tx)
Syncope + documented VT/VF
-congenital long qt
-arrhythmogenic RV cardiomyopathy
Who is Brugada?
Two terms: syndrome and pattern
One EKG: pseudo-right bbb and persistent ST elevations V1-V2
Pattern = ECG findings + ASYMPTOMATIC
Syndrome = ECG findings + at least one episode of sudden cardiac death or sustained VT
Anesthetic implications (See http://bja.oxfordjournals.org/content/89/5/788.full)
-likely has AICD, so disable it
-avoid alpha agonists
-Contraindicated / MUST avoid class 1 antiarythmics ( see: https://en.wikipedia.org/wiki/Antiarrhythmic_agent#Class_I_agents)
so NO LIDOCAINE OR PHENYTOIN!
-caution with iso? (http://www.jcvaonline.com/article/S1053-0770%2801%2907405-5/abstract)