Brugada Syndrome is more common that you might think.

Brugada Syndrome may be responsible for up to 50% of all sudden deaths that occur in individuals with apparently normal hearts. On the website maintained by the Brugada brothers who first identified the syndrome, they estimate that it may be indicated in somewhere between 4 and 12% of all sudden deaths.

A study of the ECGs of 12,000 non-cardiac patients in a large urban teaching hospital was undertaken to determine how many of them exhibited the classic Brugada Syndrome pattern. The results showed that 52 were found to have ECG patterns specifically consistent with Brugada Syndrome. The authors of this study note that Brugada Syndrome is much more prevalent than previously thought.

To put it into perspective, this means that in a high school with 2,000 students, you might expect that there are 7 students in that population who would test positive for Brugada Syndrome. Sobering information. In a future post, I'll discuss the status of routine ECG screenings of children in our country compared with other industrialized countries.

So if this is true, why haven’t we heard of it more often? Well, it is not a new disease, but it is newly diagnosed. Brugada Syndrome was first identified back in the early nineties by Drs. Josep and Pedro Brugada. It is hereditary, but having the syndrome doesn’t necessarily mean that the patient will be struck down by it. In my husband Jim’s case, he had no memory of anyone in his parents’ families dying young and healthy. It may skip a generation, or the affected relatives may have died of other causes before they might have suffered from a cardiac event brought on by Brugada Syndrome. Unfortunately, the statistics for long term survival of known Brugada patients are not great. The ICD is the only way to be absolutely sure a patient will survive sudden cardiac death.

In instances of young healthy children or adults dying with no explanation, or sudden crib death, or even episodes of fainting that can’t be explained otherwise, the survivors or patients should definitely be screened for Brugada Syndrome.

Because so many physicians in the United States have never encountered Brugada Syndrome, the onus may be on the patient to be diligent about follow-up if any of the circumstances I described above happen in your family. This is such a new syndrome, and the research is ongoing, that it’s possible for a very good physician to simply not be familiar with the disease.

(photo above is of my son Dave and daughter, Jessica, outside the White House lawn in 1990.)