Brugada Syndrome

Brugada syndrome is a potentially life-threatening heart rhythm disorder. It’s characterized by a specific abnormal heartbeat, detected by an electrocardiogram test, called a Brugada sign. Brugada syndrome is frequently an inherited condition.

Many people who have Brugada syndrome don’t have any symptoms, and so they’re unaware of their condition. For some, however, Brugada syndrome can cause dangerous irregular heart rhythms that can cause fainting or sudden cardiac arrest.

Brugada syndrome is treatable using an implanted medical device called an implantable cardioverter-defibrillator. Because Brugada syndrome was discovered recently, research is still under way on other treatments for the disorder.

Symptoms

Many people who have Brugada syndrome are undiagnosed because the condition often hasn’t caused any noticeable signs or symptoms.

The most important sign or symptom of Brugada syndrome is an abnormal heart rhythm (arrhythmia) called a Brugada sign. A Brugada sign is a pattern of heartbeats that’s found on a test of your heart rhythm (electrocardiogram, or ECG). You can’t feel a Brugada sign — it’s only detected on an ECG.

It’s possible to have a Brugada sign without having Brugada syndrome. However, signs and symptoms that could mean you have Brugada syndrome include:

• Fainting (syncope)
• Irregular heartbeats or palpitations
• Stopped heartbeat (sudden cardiac arrest)

Brugada syndrome signs and symptoms are similar to some other heart rhythm problems, so it’s essential that you see your doctor to find out if Brugada syndrome or another heart rhythm problem is causing your symptoms.

When to see a doctor

If you have heart palpitations or an irregular heartbeat (arrhythmia), make an appointment to see your doctor. Your problem could be caused by a heart rhythm problem, but tests will be necessary to see if your heart problem is Brugada syndrome. If you faint and you suspect it may be because of a heart condition, seek emergency medical attention.

If your parent, sibling or child has been diagnosed with Brugada syndrome, you may also want to make an appointment with your doctor. He or she can discuss whether you should undergo genetic testing to see if you’re at risk of Brugada syndrome.

Causes

Brugada syndrome is a heart rhythm disorder. Each beat of your heart is triggered by an electrical impulse generated by special cells in the right upper chamber of your heart. Tiny pores, called channels, on each of these cells direct this electrical activity, which makes your heart beat. In Brugada syndrome, a defect in these channels can cause your heart to beat abnormally.

During these episodes, your heart doesn’t pump effectively. As a result, not enough blood travels to the rest of your body. This can cause fainting, other heart rhythm disorders, or in extreme cases, sudden cardiac death.

Brugada syndrome is usually inherited, but it may also result from a structural abnormality in your heart, imbalances in chemicals that help transmit electrical signals through your body (electrolytes), or the effects of certain prescription medications or cocaine use.

Brugada syndrome is usually diagnosed in adolescents and adults. It’s rarely diagnosed in young children.

Risk factors for Brugada syndrome include:

• Family history of Brugada syndrome. If other family members have had Brugada syndrome, you’re at an increased risk of having the condition.
• Being male. Adult men are more frequently diagnosed than are women. In young children and adolescents, however, boys and girls are diagnosed at about the same rate.
• Race. Brugada syndrome occurs more frequently in Asians than in other races.
• Fever. While having a fever doesn’t bring on Brugada syndrome itself, fever can increase the risk of fainting or other complications of Brugada syndrome, especially in children.

Complications

The most serious complication of Brugada syndrome is sudden cardiac arrest. Sudden cardiac arrest is the sudden, unexpected loss of heart function, breathing and consciousness. It’s a medical emergency. If not treated immediately, the condition is fatal, resulting in sudden cardiac death. With fast, appropriate medical care, survival is possible. Administering cardiopulmonary resuscitation (CPR) — or even just rapid compressions to the chest — can improve the chances of survival until emergency personnel arrive.

Sudden fainting (syncope) is another complication of Brugada syndrome. If you have Brugada syndrome and you faint, seek emergency medical attention.

Tests

Aside from a typical physical examination and listening to your heart with a stethoscope, tests to see if you have Brugada syndrome may include an Electrocardiogram (ECG) with medication, Electrophysiology (EP) test and Genetic testing.

Treatments

Treatment of Brugada syndrome depends on the risk of an abnormal heartbeat (arrhythmia). Because of the nature of the heart rhythm abnormality, medications can’t treat Brugada syndrome — only a medical device called an implantable cardioverter-defibrillator can.

Implanting the device is usually recommended for people at high risk of sudden cardiac death or other complications of Brugada syndrome. This small device continuously monitors your heart rhythm and delivers electrical shocks when needed to control abnormal heartbeats. The procedure to implant an ICD requires hospitalization for a day or two.

There’s a risk of complications from having an ICD implanted. People who have an ICD implanted as a treatment for Brugada syndrome have reported receiving shocks from their ICD even when their heartbeat was regular. This may be because many people who receive an ICD as a treatment for Brugada syndrome are young, and may receive shocks when their heart rates increase during normal stresses, such as exercise. Your doctor will program your ICD to reduce this risk. If you have an ICD implanted as part of your Brugada syndrome treatment, talk to your doctor about ways to avoid inappropriate shocks.

Source: MayoClinic.com