Health Library

Brugada Syndrome in Children

Brugada syndrome in children is usually inherited, meaning it can run in families. It is more common in males and people of Southeast Asian descent. In rare cases, it can develop later in life.

It is identified by a pattern seen on an electrocardiogram (ECG) or by genetic testing.

Early diagnosis is important. It can help prevent serious problems, including sudden cardiac arrest.

How the Heart’s Electrical System Normally Works

The heart has its own electrical system. This system controls how the heart beats. Electrical signals travel through heart cells. These signals tell the heart when to squeeze and relax.

Sodium channels help these electrical signals move from cell to cell. They act like tiny doors in heart cells. When the heart is at rest, the sodium channel is closed, keeping sodium ions outside the cell.

An electrical signal tells the gate to open. Sodium rushes into the cell, which triggers a heartbeat.

Almost right away, part of the channel blocks the opening, stopping the flow of the sodium. The channel then closes and resets for the next heartbeat.

When this system works well, the heart beats in a steady rhythm. If the signals are disrupted, it can cause arrhythmias (abnormal heart rhythms).

What Happens in Brugada Syndrome?

In Brugada syndrome, sodium channels do not work as they should. This disrupts how electrical signals move through the heart.

As a result, the heart can develop dangerous rhythms, such as ventricular tachycardia and ventricular fibrillation. These abnormal rhythms can start suddenly, often during rest or sleep.

Doctors can often see this pattern on an electrocardiogram. This is called a Brugada ECG pattern.

Some children have no symptoms, while others may have serious events without warning.

What Causes Brugada Syndrome?

Most cases are caused by changes in a gene. The most common gene is called SCN5A, which affects how heart cells send electrical signals.

Brugada syndrome is often inherited in an autosomal dominant way. This means a child can inherit the condition from one parent who has the SCN5A gene.

In some cases, no gene is found, but the condition can still happen.

Certain triggers can make Brugada syndrome more likely to appear or worsen symptoms. These include:

• Fever, especially above 100.4ºF
• Medications, such as certain antidepressants or anesthetics
• Alcohol, especially heavy or binge drinking
• Electrolyte imbalances (low potassium or calcium, or high calcium)

Fever is a common trigger in children and can increase the risk of dangerous arrhythmias.

Signs and Symptoms Parents Should Watch For

Symptoms of Brugada syndrome can vary. Some children have no symptoms at all.

Possible symptoms include:

• Fainting (syncope)
• Gasping or labored breathing during sleep
• Dizziness
• Seizures
• A fluttering sensation in the chest (palpitations)
• Sudden cardiac arrest (heart stops beating)

Fainting in a child with no clear cause should always be checked by a doctor.

How Do Doctors Diagnose Brugada Syndrome?

A Brugada syndrome diagnosis involves several tests. An electrocardiogram (ECG) is often the first step. This test measures the heart’s electrical signals using sensors on the chest, arms and legs. It creates a graph that shows how the heart is working. Doctors look for changes linked to the Brugada pattern.

Sometimes, this pattern does not show up right away. Fever or certain medications may be used to help reveal changes on the ECG.

Doctors may also use other tests to learn more about your child’s heart rhythm, including:

• Holter monitor – tracks heart rhythm over 24 to 48 hours during daily life
• Event monitor – worn longer and records heart rhythm during symptoms
• Family history review – looks for patterns of fainting, sudden death or heart rhythm problems in relatives
• Genetic testing for inherited forms – looks for gene changes that cause Brugada syndrome

Because this condition can run in families, other relatives may also need testing.

What are Treatment Options for Brugada Syndrome?

Brugada syndrome treatment in children depends on your child’s risk level. Some children may only need close monitoring over time.

Daily care focuses on lowering risk. This includes treating fevers quickly and avoiding medications that can trigger arrhythmias.

For children at higher risk, more advanced treatment may be needed, like:

• Implantable cardioverter-defibrillator (ICD) – small device placed under the skin that detects and resets dangerous heart rhythms
• Medicines – such as quinidine to help control the heart rhythm
• Ablation – this is a newer procedure to “burn” away the area of the heart most likely to cause arrhythmias. It is mainly used in higher-risk children and adults who cannot tolerate medications or where ICDs are not helpful.

Living With Brugada Syndrome

Children with Brugada syndrome can live active, full lives with the right care.

Daily habits play an important role in managing Brugada syndrome. Families should:

• Treat fevers right away
• Avoid known triggers
• Review safe medications with their care team

It is also important to plan for everyday activities. Talk with your child’s care team about sports and activities.

Schools should have an emergency plan. Access to an AED (automated external defibrillator) can be lifesaving.

Since Brugada syndrome is often inherited, genetic counseling and family screening are important.

Recovery and Ongoing Care

Brugada syndrome requires lifelong care and monitoring. Children need regular follow-up with a heart rhythm specialist. Doctors will check your child’s heart rhythm over time.

As your child grows, their care plans may change. This helps match their needs at each stage of life. Ongoing care helps lower the risk of serious heart rhythm problems.

What is the Long-Term Outlook for Children With Brugada Syndrome?

The outlook for children with Brugada syndrome depends on the symptoms and risk level. With early diagnosis and proper care, many children do well. Treatment can lower the risk of sudden cardiac death.

Most children have a good quality of life with the right support and planning.

As children grow into adults, they will transition to adult heart rhythm care. This ensures continued monitoring and support over time.

Frequently Asked Questions

Is Brugada syndrome life-threatening?

It can be. Some children are at risk for dangerous heart rhythms. Early care helps lower this risk.

Can Brugada syndrome be cured?

There is no cure. Treatment focuses on preventing serious heart rhythm problems.

Should my child avoid sports?

Not always. Activity plans depend on risk. Talk with your child’s care team.

Should family members be tested?

Yes. Brugada syndrome is often inherited. Family screening is important.

How is fever managed in children with Brugada syndrome?

Treat fever quickly with medication. Contact your care team if the fever is high (100.4^°F or higher) or does not improve.