Brugada syndrome

What happens in this disease?

The heart beats regularly thanks to electrical signals. These signals are carried by tiny channels in the heart cells. In Brugada syndrome, one of these channels does not work well. An irregular, fast rhythm can develop in the lower chambers of the heart. The structure of the heart is usually normal.

Which symptoms can occur?

Many people have no symptoms at all. Symptoms usually begin in adulthood and are more common in men.

• Fainting
• Palpitations, an irregular heartbeat
• Shortness of breath
• Seizure-like episodes
• Symptoms appearing at rest or during sleep
• Symptoms getting worse during an illness with fever

How is it diagnosed?

The diagnosis is based on a heart tracing (ECG), which shows a typical pattern. If needed, a medicine test that brings out this pattern is done under a doctor's supervision. The doctor asks about the family history. A genetic test may be done, but in many people no gene is found.

Treatment and care

There is no treatment yet that removes the disease. If the risk is high, a pacemaker-like device is placed under the skin of the chest. This device detects a dangerous rhythm and corrects the heartbeat. Some medicines and high fever can disturb the rhythm; the doctor gives guidance about these. People without symptoms are followed regularly. The doctor who follows the disease decides which treatment is suitable.

For the family: inheritance and genetic counselling

The disease is usually passed on through dominant inheritance. A single changed gene is enough. For each child of an affected parent, the chance of passing it on is 1 in 2. Sometimes the change appears for the first time in the person. Genetic counselling guides the family.