BRUGADA SYNDROME

What is Brugada syndrome?

Brugada syndrome is a rare condition that causes an abnormal heart rhythm in your heart’s lower chambers (ventricles). This irregular heartbeat can cause fainting (syncope) and lead to sudden cardiac death (SCD). This often happens while you’re at rest or asleep.

Cardiologists identified Brugada syndrome in 1992. Researchers continue to study the condition and test new treatments.

Is Brugada syndrome dangerous?

Potentially. It can cause a dangerous heart rhythm called ventricular fibrillation, which prevents your heart from pumping blood to your brain. This is why people with Brugada syndrome are at risk of fainting and sudden cardiac death. Out of all the SCDs that happen, researchers blame Brugada syndrome for 4% of them.

Who does Brugada syndrome affect?

Brugada syndrome is more common in people assigned male at birth. Men are eight to 10 times more likely than women to have the condition. Anyone with a family history of SCD or Brugada syndrome should find out if they have the disease. Symptoms often start when you’re in your 30s or 40s.

The condition appears to be more common in Japan and Southeast Asia.

Is Brugada syndrome rare?

Yes, Brugada syndrome is rare. An estimated 5 people out of 10,000 have the condition.

What are the symptoms?

Brugada syndrome symptoms can happen at any age and may include:

  • Ventricular arrhythmia (an irregular heart rhythm that begins in the lower chambers of your heart).
  • Fainting (syncope).
  • Dizziness.
  • Difficulty breathing.
  • Seizures.
  • Heart palpitations (can feel like a fluttering or a flip-flopping in your chest).
  • Atrial fibrillation (a fast, irregular heart rhythm that starts in your heart’s upper chambers).
  • Cardiac arrest. This may be the first symptom and the reason some infants and children with Brugada syndrome die in their sleep.

The symptoms of Brugada syndrome are similar to many other conditions. It’s important to see a healthcare provider to find out what’s causing your symptoms.

More than 70% of people with Brugada syndrome don’t have any symptoms. Sometimes providers find the condition during an electrocardiogram (ECG). An EKG is a recording of the heart’s electrical activity. People with Brugada syndrome often have a recognizable pattern (Brugada pattern) on the EKG printout.

What triggers Brugada syndrome?

A fever can make Brugada symptoms start. This is why people with Brugada syndrome need to treat a fever right away — even if they have an implantable cardioverter defibrillator (ICD). You can take over-the-counter medicines to bring your fever down.

Other symptom triggers include:

  • Heat exhaustion.
  • Dehydration.
  • Sodium channel blockers (medicines that block sodium).
  • Lithium.
  • Tricyclic antidepressants.
  • Drinking too much alcohol.
  • Cocaine.
  • Marijuana.

What is the cause of Brugada syndrome?

About 70% of people with Brugada syndrome don’t have a known genetic mutation. However, others have a genetic mutation in one of 18 or more genes. These mutations interfere with heartbeat signal conduction in your heart.

People without a genetic mutation may get Brugada syndrome from an unknown cause or from medicines they take for certain mental health conditions or heart issues.

How is Brugada syndrome inherited?

It only takes one copy of an affected gene from one parent to inherit Brugada syndrome. Any child of someone with a Brugada-related gene mutation has a 50% chance of having it too.

Do I (or does my family) need a screening for Brugada syndrome?

Brugada syndrome is a medical condition that one generation can pass to the next. It’s important to schedule a screening for this condition if you have a first-degree relative with Brugada syndrome. First-degree relatives are your parents, siblings and children.

The first step is to tell your provider that you have a family history of Brugada syndrome. They may want to do diagnostic tests to check your heart. If these tests are positive, you should see a cardiologist who’s familiar with this condition.

How is Brugada syndrome diagnosed?

To make a Brugada syndrome diagnosis, a healthcare provider will:

  • Perform a physical exam.
  • Review your medical history.
  • Review your family history, especially a history of sudden cardiac death.
  • Order tests.

What tests will be done to diagnose Brugada syndrome?

Tests for diagnosing Brugada syndrome include:

  • Genetic testing: This can confirm that you have a specific genetic mutation that indicates Brugada syndrome. Other family members may want to have a test for this mutation.
  • Electrocardiogram (ECG): This test records the electrical activity that makes your heart beat. Because a pattern typical of Brugada syndrome may not appear at first, a provider may immediately do the test two more times. They reposition the leads between tests to try to capture the pattern. They also may do an EKG before and after you eat a large meal.
  • ECG with medication: Before doing an EKG, your provider may give you medication that helps the Brugada pattern show up on the EKG printout if you have the condition. Your provider may call this a drug challenge test.

Based on your ECG results, you may also have:

  • Electrophysiology (EP) testing: Providers place catheters inside your heart through your femoral (leg) veins. These catheters measure electrical activity from inside your heart. You may have this test if your diagnosis is unclear after earlier tests.
  • Lab tests: These check for a normal potassium-calcium balance and can rule out other causes of an abnormal heart rhythm.

How is it treated?

Since there isn’t a cure, the goal of Brugada syndrome treatment is to keep you from having ventricular arrhythmias and treat them when they happen.

Your treatment may include:

  • Isoproterenol.
  • Quinidine.
  • Amiodarone.
  • Implantable cardioverter defibrillator (ICD) if you’re at a high risk for ventricular fibrillation, have fainting spells or survived a cardiac arrest.
  • Ablation (in some cases).

If you’re not having symptoms, your provider may decide you need an ICD because of your family history or test results. Some providers may do frequent follow-ups and only treat you when you have symptoms. However, others don’t agree with this approach because your first symptom could be sudden cardiac death.

Complications of the treatment

ICDs aren’t perfect devices. They can:

  • Deliver a shock at the wrong time.
  • Have a malfunctioning lead.
  • Harbor an infection.

How long does it take to recover from this treatment?

You can do many activities a few days after receiving an ICD, but you’ll likely wait a week to drive. You can exercise a little every day, but not to the point of tiring yourself out. Don’t do any heavy lifting or strenuous activities until your provider tells you it’s OK.

How can I reduce my risk?

If you inherited Brugada syndrome from a parent, you can’t change that. However, if you know you have Brugada syndrome or it’s in your family, you and your relatives can get a genetic test to check for it. Also, people considering pregnancy can see a genetic counselor to find out if they’re at risk of passing it on to their children.

What can I expect if I have Brugada syndrome?

There’s no cure for Brugada syndrome, but treatments are available to lower your risk for SCD. Also, researchers are testing ablation to see if that can help people with this condition. People with Brugada syndrome can prevent symptoms by avoiding certain medications or substances.

People with Brugada syndrome who have symptoms but aren’t receiving treatment have a high risk of SCD. People without symptoms and with a normal EKG have a much lower risk of SCD.

How do I take care of myself?

If a provider diagnosed you with Brugada syndrome, an experienced healthcare team should evaluate and treat you. This approach helps ensure that you get the best possible care. The team should include the following:

  • Electrophysiologist: A provider who specializes in diagnosing and treating patients with heart rhythm problems.
  • Genetic counselor: A specialist who studies genetic patterns to identify gene defects that can cause problems.
  • Primary care physician: Your “regular” provider who can provide general follow-up care and monitor your medications.

Avoid the things that trigger Brugada syndrome symptoms and be sure to tell your provider about anything unusual.

When should I see my healthcare provider?

You should have an appointment with your provider at least once a year. If you have an ICD, your provider should check your device at least twice a year.

When should I go to the ER?

You need immediate medical care if you’re in cardiac arrest. Since you won’t be able to call for help yourself if this happens, someone near you will need to help. If you’re at risk of cardiac arrest, ask your family to get CPR training and call 911 or a local emergency number. You can also let co-workers know about your risk in case you need their help.

What questions should I ask my doctor?

Ask your provider:

  • Do I need an ICD?
  • What kind of maintenance does an ICD need?
  • Can you recommend a support group I could join with others who have this condition?

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