Brugada syndrome is a hereditary condition which affects the electrical conduction system of the heart, causing an abnormal heart rhythm. In some cases, Brugada syndrome can be life-threatening, and may come about with little warning, resulting in sudden death.
With every normal heartbeat, an electrical signal courses through the heart in a highly controlled fashion. Each portion of the heart must electrically activate, then reset to allow the next beat to follow. The proteins that regulate this process, called ion channels, regulate the flow of sodium, potassium, chloride, calcium, magnesium, and other molecules across heart cells.
When an electrocardiogram (ECG or EKG) is performed on a patient, the heart’s electrical activation and reset are being recorded and evaluated. Activation of each section of the heart is seen on the ECG: the P wave represents atrial activation, the QRS represents left and right ventricular activation, and the QT interval and T wave represent the resetting of the left and right ventricles.
Brugada syndrome was first described in 1992 in a series of patients with sudden death who had similar, peculiar electrocardiogram (ECG) abnormalities. It was later found that many of these patients had abnormal function of their sodium ion channels.
Patients with Brugada syndrome develop ventricular tachycardia, a condition where the heart beats too quickly to maintain normal blood flow. Brugada Syndrome is rare. Although the number of patients with the condition is difficult to measure, approximately 4 out of 1,000 people in the U.S. have ECG findings of Brugada syndrome.
The condition is found more commonly in men and although more common in those of Asian descent, those of European background can also be affected. The average age at diagnosis is 42, but the condition can present itself at any age.