Brugada (brew-GAH-dah) syndrome is a rare, but potentially life-threatening heart rhythm disorder that is sometimes inherited.

People with Brugada syndrome have an increased risk of having irregular heart rhythms beginning in the lower chambers of the heart (ventricles).

Brugada syndrome treatments include preventive measures such as avoiding aggravating medications and reducing fever. When necessary, treatment may also include a medical device called an implantable cardioverter-defibrillator (ICD).

Many people with Brugada syndrome don't know they have the condition. Brugada syndrome often doesn't cause any noticeable symptoms.

Signs and symptoms that may be associated with Brugada syndrome include:

• Dizziness
• Fainting
• Gasping, labored breathing, particularly at night
• Irregular heartbeats or palpitations
• Extremely fast and chaotic heartbeat
• Seizures

A major sign of Brugada syndrome is an abnormal result on an electrocardiogram (ECG), a test that measures the electrical activity of the heart.

When to see a doctor

Brugada syndrome signs and symptoms are similar to some other heart rhythm problems. It's important that you see your doctor to find out if Brugada syndrome or another heart rhythm problem is causing your symptoms.

If you faint and you think 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 ask your doctor if you should have testing to see if you're at risk of Brugada syndrome.

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 causes your heart to beat abnormally fast, creating a dangerous heart rhythm (ventricular fibrillation).

As a result, your heart doesn't pump enough blood to the rest of your body. You can faint if the abnormal rhythm lasts for a short time. Sudden cardiac death can occur if the abnormal heart beat doesn't stop.

Brugada syndrome may be caused by:

• Structural abnormality in your heart, which may be hard to detect
• An imbalance in chemicals that help send electrical signals through your body (electrolytes)
• Use of certain prescription medications or cocaine

Risk factors for Brugada syndrome include:

• Family history of Brugada syndrome. This condition is often passed down through families (inherited). If other family members have had Brugada syndrome, you're at an increased risk of having it, too.
• Being male. Men are more frequently diagnosed with Brugada syndrome than are women.
• Race. Brugada syndrome occurs more frequently in Asians than in other races.
• Fever. A fever doesn't cause Brugada syndrome, but it can irritate the heart and trigger fainting or sudden cardiac arrest in someone with Brugada syndrome, especially in children.

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.
• Fainting. If you have Brugada syndrome and you faint, get emergency medical attention.

If someone in your family has Brugada syndrome, genetic testing can be done to determine if you have, or are at risk of, the condition.

Brugada syndrome usually is diagnosed in adults and, sometimes, in adolescents. It's rarely diagnosed in young children because the symptoms are often unnoticed.

To diagnose Brugada syndrome, your doctor will perform a physical exam and listen to your heart with a stethoscope. Tests are done to check your heart beat and diagnose or confirm Brugada syndrome.

• Electrocardiogram (ECG) with or without medication. An ECG is a quick, painless test that records the electrical signals in your heart. During an ECG, sensors (electrodes) are attached to your chest and sometimes your limbs. The test can help your doctor detect problems with your heart's rhythm and structure.

  If your heart beat is normal during the test, your doctor may order a portable ECG that you wear during your daily activities for a full day and night. This type of test is called a 24-hour Holter monitor.

  If you have symptoms of Brugada syndrome, but your initial ECG and 24-hour Holter test were normal, your doctor may give you medications through an IV that may trigger an abnormal heart beat.

• Echocardiogram. An echocardiogram uses sound waves to create images of your heart. This test cannot diagnose Brugada syndrome on its own, but it can help your doctor rule out any structural problems with your heart.

• Electrophysiology test. If your ECG shows signs of Brugada syndrome, or if you have had symptoms such as sudden cardiac arrest, your doctor may recommend an electrophysiology test to see how easy it is to get the heart to go into the abnormal Brugada rhythm.

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

Brugada syndrome treatment depends on your risk of a serious abnormal heartbeat.

You're considered at high risk if you have:

• A personal history of serious heart rhythm problems
• Fainting spells
• Survived sudden cardiac arrest

If you don't have any symptoms, you may not need any specific treatment because your risk is likely low. However, your doctor will recommend the following steps to reduce your chances of an abnormal heart rhythm.

• Treat a fever aggressively. Fever is a known trigger of abnormal heartbeats in people with Brugada syndrome, so use fever-reducing medications at the first sign of a fever.
• Avoiding drugs that may trigger an abnormal heart rhythm. Many drugs can increase the risk of an irregular heartbeat, including certain heart medications and antidepressants. Too much alcohol can also increase your risk. Always tell your doctor about the medications you take, including drugs and supplements bought without a prescription.
• Avoiding playing competitive sports. If you're at high risk of a serious irregular heart rhythm, your doctor may tell you not to play competitive sports.

Surgery or other procedures

If you've had cardiac arrest or a worrisome fainting episode, the main treatment is an implantable cardioverter-defibrillator (ICD).

• Implantable cardioverter-defibrillator (ICD). This small, battery-operated device is placed in your chest to continuously monitor your heart rhythm and delivers electrical shocks when needed to control abnormal heartbeats. ICD placement usually requires an overnight hospital stay. ICDs may cause unnecessary shocks when they're not needed, so it's important to discuss the benefits and risks with your doctor.
• Drug therapy. Sometimes, medications such as quinidine are used to prevent a potentially dangerous heart rhythm. This drug may be given along with an ICD.
• Catheter ablation. If an ICD doesn't effectively and safely control your Brugada syndrome symptoms, a procedure called radiofrequency catheter ablation may be an option. A long, flexible tube (catheter) is inserted through a blood vessel and threaded to your heart. The catheter delivers high energy that scars or destroys the heart tissue responsible for the abnormal heart rhythm.

If you have Brugada syndrome, it's important to schedule regular visits 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.

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.

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.

If your doctor thinks you have Brugada syndrome, you'll likely need several appointments to confirm the diagnosis and figure out how serious your condition is. Your doctor should give you instructions before each visit on how you need to prepare for the other appointments.

Here's some information to help you get ready for your appointment, and to know what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance. For example, if you're having a test to look at your heart's electrical activity (electrophysiology test), ask your doctor how long you need to fast before your test.
• Write down any symptoms you're experiencing, including any that may seem unrelated to Brugada syndrome.
• Write down key personal information, especially any family history of sudden death, cardiac arrest or heart conditions, and any personal history of fainting or heart arrhythmias.
• Make a list of all medications, vitamins or supplements you're taking.
• Take a family member or friend along, if possible. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions for your doctor.

Preparing a list of questions will help you make the most of your time with your doctor. For Brugada syndrome, some basic questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• What kinds of tests do I need?
• What types of treatments are available for Brugada syndrome? Which do you recommend for me?
• What's an appropriate level of physical activity?
• How often do I need follow-up visits to monitor my condition?
• I have other health conditions. How can I best manage these conditions together?
• Are there brochures or other printed material I can take home with me? What websites do you recommend?
• Should my family be screened?

Don't hesitate to ask any other questions you have.

What to expect from your doctor

Your doctor is likely to ask you a number of questions, such as:

• Do you have a family history of Brugada syndrome or other heart rhythm problems?
• When did your symptoms start?
• Have your symptoms been continuous or occasional?
• How often do you have symptoms, such as fainting?