Introduction

Brugada syndrome is an abnormality in the heart's electrical system that causes life-threatening heart rhythm disturbances (arrhythmias). It was first described just over 10 years ago by 3 brothers who are all cardiologists, after whom the condition was named. It is diagnosed by specific abnormalities on the electrocardiogram (ECG).

Each beat of the heart is triggered by an electrical impulse from special cells (called the sinus node) in the right upper chamber of your heart (called the atrium). Tiny pores, called ion channels, on each of these cells direct this electrical activity. In the Brugada syndrome, a defect in these channels causes episodes of abnormal electrical function leading to arrhythmias.

The Brugada syndrome may be a hereditary condition ie. passed down in families.

How does it manifest

A common presentation is syncope (fainting spells) due to transient episodes of abnormal heart rhythm. During these episodes, the pumping function of the heart is impaired. This decreases blood flow to the brain, causing fainting. In its most extreme/dramatic form, it may also lead to chaotic, uncoordinated electrical activity (ventricular fibrillation), which causes the heart to quiver and stop pumping blood. Sudden death usually follows — unless the heart receives an immediate electrical shock from a device called a defibrillator.

However, most individuals with the Brugada syndrome may have no symptoms at all.

Who are at risk

Though it occurs most often in young adults, it has been documented in all ages.
Those at high risk are those with: (1) family history of sudden cardiac death; (2) personal history of serious heart rhythm problems, and (3) personal history of severe fainting spells.

Brugada syndrome may be inherited, and certain genes have been found to be associated. If someone is diagnosed to have Brugada syndrome, other family members may be requested to perform an ECG test to see if they also have the features of the condition.

There is no known cure for the condition at this time.