What is Brugada Syndrome?

This is a rare but serious condition. It affects your heart's normal rhythm and can make it beat too fast or in an irregular way. When that happens, it's called an arrhythmia. With an arrhythmia, your heart can't pump blood to the rest of your body as it's supposed to.

Brugada syndrome is one of the most common causes of heart-related sudden death in people who are otherwise young and healthy. It affects about 5 in 10,000 people worldwide. It's most common in people of Japanese and South Asian descent and happens much more often in men.

Symptoms

See your doctor if you have:

• Difficulty breathing
• Fainting
• Irregular heartbeat
• Seizures

If you have Brugada syndrome, a high fever can bring on these symptoms or make them worse.

Causes

It can run in families. About 30% of people who have it have a problem with a gene that helps their heart stay in normal rhythm. If you have a family member who has it, you may want to see your doctor to find out if you're at risk for it too.

In other cases, doctors don't know what causes it. Some possibilities include:

• Cocaine use
• High levels of calcium in your blood
• Medicines that treat high blood pressure, depression, or chest pain
• Very high or very low levels of potassium

Diagnosis

If your doctor thinks you might have Brugada syndrome, they'll recommend a physical exam along with some other tests:

• Electrocardiogram (EKG or ECG): This test records the electrical activity of your heart to find out if there's a problem with its rhythm. A technician will put electrodes (small patches with wires) on your chest that pick up and record electrical signals from your heart. You also might take medication -- usually given through an IV -- that will help identify a certain pattern caused by Brugada syndrome.
• Electrophysiology studies (EPS): If an EKG shows you have Brugada syndrome, this test can help your doctor see where the arrhythmia is coming from and understand how to treat it. You'll be given some medication to make you sleepy. Then they'll put a flexible tube (called a catheter) through a vein in your groin and up to your heart. Electric signals are sent through the catheter, and they record what's happening in the different areas.
• Genetic testing: A sample of your blood is tested to see if you have the gene that may cause it.

Treatment

If you're diagnosed with the syndrome, your doctor will likely recommend a small device called an implantable cardiac defibrillator (ICD) that's similar to a pacemaker. It monitors your heart's rhythm. If it picks up an unusual heartbeat, it sends an electrical shock to correct it. The purpose is to shock your heart out of a dangerous rhythm that can cause sudden cardiac death.

Your doctor will put a flexible wire, called a lead, into a major vein near your collarbone and guide it to your heart. The ends of the lead attach to the bottom chambers of your heart. The other ends attach to a shock generator. Your doctor will implant this part of the device under your skin just below your collarbone. You may need to stay in the hospital for 1 or 2 days.

Medicine is sometimes used to treat Brugada syndrome. Your doctor may prescribe quinidine to help keep your heart rhythm normal. Some people who have an ICD also take medicine.

You'll need regular checkups to make sure you don't need to make any changes to your treatment. Your doctor also will check for any new problems that could affect your heart.

Read more on: heart disease, atrial fibrillation