Overview

Brugada (brew-GAH-dah) syndrome is a potentially life-threatening heart rhythm disorder that is sometimes inherited. People with Brugada syndrome have an increased risk of abnormal heart rhythms from the lower chambers of the heart (ventricular arrhythmias).

Many people who have Brugada syndrome don't have any symptoms, and so they're unaware of their condition. A telltale abnormality — called a type 1 Brugada ECG pattern — is detected by an electrocardiogram (ECG) test. Brugada syndrome is much more common in men than women.

Brugada syndrome is treatable with preventive measures such as avoiding aggravating medications, reducing fever and, when necessary, using a medical device called an implantable cardioverter-defibrillator (ICD).

Source: http://www.mayoclinic.com

Symptoms

Many people who have Brugada syndrome are undiagnosed because the condition often doesn't cause any noticeable symptoms.

The most important sign of Brugada syndrome is an abnormal pattern on an electrocardiogram (ECG) called a type 1 Brugada ECG pattern. You can't feel a Brugada sign — it's only detected on an ECG.

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

• Dizziness
• Fainting (syncope)
• Gasping, labored breathing, particularly at night
• Irregular heartbeats or palpitations
• Extremely fast and chaotic 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 can determine if the underlying cause of 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 want to make an appointment with your doctor. He or she can discuss whether you should have genetic testing to see if you're at risk of Brugada syndrome.

Source: http://www.mayoclinic.com

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 and spin electrically out of control in an abnormally fast and dangerous rhythm (ventricular fibrillation).

As a result, your heart doesn't pump effectively and not enough blood travels to the rest of your body. This will cause fainting if that rhythm lasts for only a short time or sudden cardiac death if the heart remains in that bad rhythm.

Brugada syndrome is often inherited, but it may also result from a hard-to-detect 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 usually is diagnosed in adults and, sometimes, in adolescents. It's rarely diagnosed in young children.

Source: http://www.mayoclinic.com

Diagnosis

Aside from a typical physical examination, listening to your heart with a stethoscope and the standard 12-lead ECG, other tests to see if you have Brugada syndrome include:

• Electrocardiogram (ECG) with medication. In this noninvasive test, a technician places probes on your chest that record the electrical impulses that make your heart beat. An ECG records these electrical signals and can help your doctor detect irregularities in your heart's rhythm and structure.

  However, because your heart rhythm can change, an electrocardiogram by itself may not detect an abnormal heart rhythm. Your doctor may give you certain medications — including some types of anti-anginals, antidepressants, antipsychotics or antihistamines — that can unmask the type 1 Brugada ECG pattern in people who have Brugada syndrome. The medication is usually injected by an intravenous (IV) line.

• Electrophysiology (EP) test. If your ECG suggests that you have Brugada syndrome, or if you have experienced symptoms such as sudden cardiac arrest, your doctor may also recommend an EP test in an effort to see how easy it is to get the heart to go into the abnormal Brugada rhythm.

  In an EP test, a catheter is threaded through a vein in your groin to your heart, similar to cardiac catheterization. Electrodes are then passed through the catheter to different points in your heart. The electrodes then map out any irregular heartbeats. The electrodes don't shock your heart — they just detect the electrical signals running through your heart.

• Genetic testing. While genetic testing isn't required to diagnose Brugada syndrome, your doctor may recommend genetic testing to help determine whether other family members are affected if you're diagnosed with Brugada syndrome.

Source: http://www.mayoclinic.com

Complications

Complications of Brugada syndrome require emergency medical care. They include:

• Sudden cardiac arrest. If not treated immediately, this sudden loss of heart function, breathing and consciousness, which often occurs while sleeping, is fatal. With fast, appropriate medical care, survival is possible.

  Administering cardiopulmonary resuscitation (CPR) — rapid compressions to the chest — and an external shock from an automatic external defibrillator (AED) can improve the chances of survival until emergency personnel arrive.

• Fainting (syncope). If you have Brugada syndrome and you faint, seek emergency medical attention.

Coping and support

Finding out you have Brugada syndrome can be difficult. You may worry about whether your treatment will work or if other family members could be at risk. There are ways to cope with your feelings about your condition, including:

• Support groups. Finding out that you or a loved one has heart disease can be unnerving. Turning to friends and family for support is essential, but if you find you need more help, talk to your doctor about joining a support group. You may find that talking about your concerns with others who are experiencing the same difficulties can help.
• Continued medical checkups. If you have Brugada syndrome, it's a good idea to regularly check in with your doctor to make sure you're properly managing your heart condition. Regular checkups can help your doctor decide if you need to change your treatment and may help catch any new problems early.

Source: http://www.mayoclinic.com

Risk factors

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 cause Brugada syndrome by itself, a fever can irritate the heart and stimulate a Brugada-triggered faint or sudden cardiac arrest, especially in children.

Source: http://www.mayoclinic.com