What Is Brugada Syndrome, and How Does It Develop?

Brugada syndrome affects the heart’s normal rhythm. It’s named after Dr. Ramon Brugada, the cardiologist who was the first person to describe this condition. 

The heart depends on electrical signals to know how fast and how often to beat. In Brugada syndrome, the electrical system has a defect that puts people at risk for an irregular heartbeat, called an arrhythmia. An arrhythmia is dangerous because it keeps the heart from beating normally. The syndrome is rare, affecting only 1 in 20,000 people in North America.

Here, we’ll cover the symptoms, causes, and treatments of Brugada syndrome, as well as how it’s diagnosed. 

What are the symptoms of Brugada syndrome?

People with Brugada syndrome may not have any symptoms unless they have a fever or are dehydrated. In these cases, they can experience:

• Palpitations (pounding heartbeats)

• Dizziness

• Chest pain

Many times, people find out they have Brugada syndrome only when they have a concerning event that leads them to seek medical attention, such as:

• An unexplained fainting spell 

• An unexplained seizure

• An irregular heart beat (an arrhythmia)

• A cardiac arrest 

Unlike other heart conditions that commonly cause symptoms during exercise, in Brugada syndrome, these events usually happen when someone is at rest or sleeping. 

What happens when you have an irregular heartbeat (arrhythmia)?

An irregular heartbeat affects your heart's ability to pump blood properly. If your heart can’t do its job, then blood and oxygen can’t get to vital organs like your lungs, kidney, and brain. A lack of blood flow can result in damage to these organs, or death.

Can Brugada syndrome cause sudden cardiac death?

Yes. Unfortunately, sometimes no one knows someone had Brugada syndrome until after they’ve died.

Studies estimate that Brugada syndrome causes 20% of sudden deaths in people who have no other heart problems. In some countries, Brugada syndrome is the second-leading cause of death in young people, after car accidents. 

What causes a person to develop Brugada syndrome?

A genetic mutation causes Brugada syndrome 30% of the time. This means that a person is born with Brugada syndrome. Some people with the genetic mutation have a family member who also has the mutation. 

It’s not clear why, but males are more likely to have Brugada syndrome than females

Other less common causes of Brugada syndrome include:

• Cocaine use

• High levels of potassium or calcium in the blood

• Certain medications 

How is Brugada syndrome diagnosed?

A visit with a cardiologist (heart specialist) is the first step for a diagnosis of Brugada syndrome. The cardiologist will: 

• Ask you about your symptoms. 

• Ask if anyone in your family had a sudden, unexplained death, especially before the age of 45. This can be a sign that the person had Brugada syndrome that was not diagnosed. 

• Perform an ECG/EKG and echocardiography (ultrasound of the heart). 

• Order blood tests to look at your potassium or calcium levels.

• Order genetic testing to look for genes that cause Brugada syndrome.

• Inject a medication into your vein to see if it causes an arrhythmia.

The last test is done only in a specialized, controlled environment, where a medical team is available to help right away with any issues that arise. 

How is Brugada syndrome treated?

Some people with Brugada syndrome often need an implantable cardiac defibrillator (ICD) and medication. All people with Brugada syndrome need to avoid arrhythmia triggers, like the ones listed below, and follow a cardiologist’s guidance about physical activity. 

Implantable cardiac defibrillator and medication

People with Brugada syndrome sometimes need an implantable cardiac defibrillator (ICD), which is a device similar to a pacemaker. The device is placed into the chest during an operation. The device monitors the heart’s rhythm at all times. If it senses an arrhythmia, it gives the heart an electric shock that should help the heart rhythm go back to normal. 

Some people may also need medications called quinidine and amiodarone if they have a lot of arrhythmia events despite having an ICD. 

Unfortunately, the ICD can’t prevent arrhythmias from happening in the first place. There is no medication that can prevent arrhythmias either. 

Avoiding triggers

People with Brugada syndrome are also advised to avoid things that can trigger arrhythmias, like:

• Dehydration

• Cocaine

• Excessive alcohol use

• Cannabis

• Certain medications

Fever can also trigger arrhythmias. People with Brugada syndrome need medical care right away if they have a fever so they can be started on fever-reducing medicines and other therapy.  

Safe physical activity

People with Brugada syndrome can carry out everyday activities though you are at risk of having an arrhythmia event during usual activity. But physical activity, especially competitive sports, can put people with Brugada syndrome at higher risk for an arrhythmia. It’s important to work with your cardiologist to determine what level of physical activity is safe for you.

What medications should you avoid if you have Brugada syndrome?

There are many medications that can cause dangerous arrhythmias if you have Brugada syndrome, including: 

• Lithium 

• Tricyclic antidepressants like amitriptyline and imipramine

• Propofol 

• Bupivacaine

• Procaine

A complete list of medications to avoid is available on BrugadaDrugs.org. The website also has a letter you can download or print that lists all the medications people with Brugada syndrome cannot use. Many people with Brugada syndrome keep this letter on their phone or in their wallet to give to healthcare providers in case they need emergency medical care. The letter is available in 25 languages. 

Can Brugada syndrome be cured?

Unfortunately, there is no cure for Brugada syndrome.

If you have Brugada syndrome that has caused symptoms or a medical emergency in the past, then it’s more likely you’ll have more episodes. For people who have a diagnosis of Brugada syndrome but never had symptoms, only 8% will go on to have events in the future.