A genetic disorder that causes an irregular heartbeat and increases the risk of sudden cardiac death

This syndrome is an inherited condition that causes an abnormal heart rhythm, known as arrhythmia. If left untreated, people with this genetic disorder are at increased risk for:

If you have a family history of this syndrome, make an appointment with your doctor to discuss genetic testing  to determine your and your family members’ risk.

Symptoms

There often are no noticeable symptoms; unfortunately, the first sign may be sudden cardiac arrest or death.

Other symptoms include:

  • Fainting, the most common symptom
  • Difficulty breathing, especially during rest or sleep
  • Disturbed sleep, including nightmares
  • Heart palpitations , or fluttering or pounding in your chest

  • Seizure during rest or sleep

Causes

In a healthy cardiovascular system, certain proteins control the flow of sodium, potassium, and calcium through the heart’s cells. These molecules help regulate the heart’s electrical activity. If this flow is disrupted, an irregular heartbeat develops. This means the heart can’t pump blood effectively to the rest of your body.


Medical experts have identified a genetic mutation in some of these proteins, which can lead to Brugada syndrome. The condition is more commonly found in men and people of South Asian descent.

Tests

Angiogram (Angiography)

An angiogram is a special X-ray taken while a special dye is injected through a thin, flexible tube called a catheter to detect blockages or aneurysms in blood vessels.

Chest X-ray

Chest X-rays use a small dose of radiation to create pictures of the structures inside the chest, including the lungs, heart, and chest wall.

Computerized Tomography (CT) Scan

The cardiac computed tomography scan, or cardiac CT, uses X-rays to create three-dimensional images of your heart and blood vessels.

Echocardiogram

An echocardiogram uses high-frequency sound waves to create images of your heart.

Electrocardiogram (ECG)

An electrocardiogram, also known as an ECG, measures the heart’s electrical activity.

Event Monitors

An event monitor is a small device that records the heart’s electrical activity. It’s similar to an electrocardiogram, but where an electrocardiogram takes place over a few minutes, an event monitor measures heart rhythms over a much longer time.

Holter Monitors

A Holter monitor is a small device that records the heart’s electrical activity. It’s similar to an electrocardiogram, but whereas an electrocardiogram records over a few minutes, a Holter monitor records over the course of a day or two.

Stress Tests

Stress tests are used to assess how your heart works during physical activity. There are several types of stress tests, including treadmill or bike stress tests, nuclear stress tests, stress echocardiograms, and chemically induced stress tests.

Treatments

The only current treatment for this syndrome is a medical device called an implantable cardioverter defibrillator (ICD), which monitors your heart’s rhythm and sends an electrical shock to correct it when necessary. Your doctor also may recommend avoiding certain medications and monitoring your temperature regularly to avoid and quickly reduce fevers, as they may aggravate this syndrome.

Implantable Cardioverter Defibrillator (ICD)

An implantable cardioverter defibrillator (ICD) is a device implanted below your collarbone that monitors your heart’s rhythm. When it detects an abnormal rhythm, it delivers an electrical impulse or shock to the heart to correct it.

Additional information

Genetic counseling and testing for heart conditions

Our cardiogenetics experts work to improve early detection and treatment for patients at risk for certain genetic heart conditions.

Read our Cardiovascular Performance & Outcomes Booklet

Ask MHVI

Have questions for our heart and vascular program? Email us at AskMHVI@medstar.net.