The first patient with this syndrome was seen in 1986. The patient was a three year old boy from Poland. He had presented multiple episodes of loss of consciousness and had been resuscitated multiple times by his father. The child's sister had died suddenly at age two after multiple episodes of aborted sudden death. When she died, she was receiving amiodarone and had a ventricular pacemaker implanted. The electrocardiograms of the two siblings were very similar and abnormal (picture 3).

 

The identification of two additional patients resulted in the presentation of the preliminary data at the meeting of the North American Society of Pacing and Electrophysiology (NASPE) in 1991. The first paper including 8 patients was published in 1992 . Since then, there has been an exponential increase in the number of patients recognized all over the world.

 

The recent discovery of the genetic abnormalities linked to this syndrome, points to it being a primary electrical disease, providing an important first step in the prevention and effective treatment of this form of sudden death in patients with a structurally normal heart.

 

"Strength does not come from physical capacity. It comes from an indomitable will"

 

The electrocardiograms of the two first patients ever seen.
They were siblings of age 2 and 3 at the time of the recordings.
Compare to picture1.

Several authors have previously reported electrocardiograms similar to the one presented in picture1. For the most part, these were considered variants of the normal electrocardiogram and no definitive link to sudden death was established.

 

 

In the 1980's the CDC (Center for Disease Control) in Atlanta, reported an abnormally high incidence of sudden death in young immigrants from Southeast Asia. Interestingly, the natives knew the problem for many decades.

 

 

 

 

In the Northeast of Thailand, this form of death was known as Lai Tai (death during sleep). The indigenous believe that the young men died during sleep because widow ghosts came to take them away.

 

Many young men actually dress still as women to go to sleep at night - a practice carried out for more than 70 years - with the hope that it would mislead the widow ghost, who then would not take these young men.

 

In Philippines the phenomenon was known as Bangungut (scream followed by sudden death during sleep) and in Japan as Pokkuri (unexpected sudden death at night). The incidence of this form of sudden death has been estimated between 26 and 38 cases per 100,000 inhabitants per year.

 

In Laos it may cause 1 sudden death per 1.000 inhabitants per year. Unexpected sudden death is the most common cause of natural death in young Thai people. It has been only recently discovered that many of these patients suffer the Brugada syndrome. The higher prevalence of this syndrome in some areas can be explained by its genetic transmission.