What is Brugada Syndrome? Symptoms, and Treatment

Have you ever heard of Brugada Syndrome? If not, you are not alone.

Brugada syndrome is a rare but potentially life-threatening heart condition that affects the electrical activity of the heart. It can cause irregular heartbeat, fainting, seizures, and even sudden cardiac death.

In this blog post, we will discuss the possible causes of Brugada syndrome, how it is treated, and what you can do to prevent it.

What is Brugada syndrome?

Brugada syndrome is a genetic disorder that affects the sodium channels of heart cells. These channels are responsible for controlling the flow of electrical currents that drive the heartbeat.

It is characterized by a distinctive pattern on an electrocardiogram (ECG). This pattern shows a disruption in the normal rhythm of the lower chambers of the heart (ventricles), which can lead to ventricular fibrillation, a chaotic and fast heartbeat that prevents the heart from pumping blood to the body.

It is named after Spanish cardiologists Pedro and Josep Brugada, who first described it in 1992.

It is also known as sudden unexplained nocturnal death syndrome (SUNDS), bangangut, or pokkuri death syndrome in some Asian countries where it is more prevalent.

It is rare, affecting about 5 in 10,000 people worldwide. It is more common among men and people of Asian descent, particularly Japanese and Southeast Asian ancestry.

Types of Brugada syndrome

There are three types, based on the pattern of ST-segment elevation in the electrocardiogram (ECG) in leads V1 to V3. The ST segment is the part of the ECG that shows the time between ventricular depolarization and repolarization.

Type 1 Brugada Syndrome

  1. This is the only type that is diagnostic of Brugada syndrome.
  2. It shows a coved ST-segment elevation of more than 2 mm, followed by a negative T wave.
  3. It may be present all the time or only appear intermittently or after certain triggers, such as fever, drugs, or electrolyte imbalance.
  4. It is associated with one or more of the following clinical criteria:
    • Documented ventricular fibrillation or polymorphic ventricular tachycardia.
    • Coved-type ECGs in family members.
    • Syncope (fainting).
    • Nocturnal agonal respiration (gasping for air during sleep).

Type 2 Brugada Syndrome

  1. This type is not diagnostic but may indicate a higher risk of developing type
  2. It shows a saddleback ST-segment elevation of more than 2 mm, with a positive or biphasic T wave.
  3. It may also be influenced by external factors or change over time.

Type 3 Brugada Syndrome

  1. This type is also not diagnostic but may warrant further investigation.
  2. It shows either a coved or a saddleback ST-segment elevation of less than 2 mm, with any shape of T wave.
  3. It is often difficult to distinguish from normal variants or other conditions that affect the ECG.

Related: Heart Transplant: The Ultimate Solution for End-stage Heart Failure

Signs of Brugada syndrome

Many people do not have any symptoms, but some may experience:

  • Dizziness
  • Fainting or passing out (syncope)
  • Palpitations or chest pain
  • Gasping and breathing with difficulty, especially at night
  • Very fast and irregular heartbeat (ventricular fibrillation)
  • Sudden cardiac death
  • Seizures

Symptoms of Brugada syndrome often appear at rest or during sleep. They may be triggered by factors such as fever, certain drugs or drugs (e.g., cocaine), electrolyte imbalance, alcohol consumption, or strenuous physical activity.

Causes of Brugada syndrome

The most common cause is a mutation in the SCN5A gene, which codes for a protein that forms sodium channels in the heart muscle cells. These channels are responsible for regulating the electrical activity of the heart and maintaining a normal heartbeat. When there is a defect in these channels, it can result in abnormal electrical signals that trigger ventricular fibrillation.

Other genes associated with it include SCN1B, SCN2B, SCN3B, SCN4B, GPD1L, CACNA1C, CACNB2B, KCNE3, KCND3, KCNH2, RANGRF, MOG1, HCN4, SLMAP, ABCC9, and PKP2.

In some cases, it may be caused by other factors that affect the electrical activity of the heart, such as:

  1. A structural problem in the heart.
  2. An imbalance in electrolytes, such as potassium, calcium, or magnesium.
  3. Certain medications or drugs that block sodium channels or affect other aspects of cardiac function.
  4. Fever or infection that raises body temperature

How to diagnose Brugada syndrome?

The diagnosis is based on the presence of the characteristic ECG pattern and the clinical history of the patient and their family.

1. Genetic testing.

It may be performed to identify the SCN5A mutation or other genetic variants that may be associated with it. This can help confirm the diagnosis and provide information for family screening and counseling.

2. Magnetic resonance imaging (MRI).

To check for structural issues in the heart.

3. Electrophysiology (EP) test.

To measure the electrical activity and conduction pathways of the heart.

4. Drug challenge test.

This involves giving a drug that blocks sodium channels, such as ajmaline or flecainide, and observing changes in the ECG. This test can uncover Brugada syndrome in people who have a normal ECG at rest.

Risk factors for Brugada syndrome

Some risk factors that may increase the likelihood or severity include:

  • History of Brugada syndrome or sudden cardiac death in the family
  • Male gender
  • Asian descent
  • The use of certain drugs or drugs that block sodium channels (for example, antiarrhythmics, antidepressants, antihistamines, etc.)
  • Fever or infection
  • Electrolyte imbalance (for example, low potassium or calcium levels)
  • Alcohol consumption
  • Vigorous physical activity
  • Abnormal heart structure or function

Treatment for Brugada syndrome

The main goal of treatment is to prevent sudden cardiac death due to ventricular fibrillation. Treatment options may vary depending on the type and severity of the condition, the presence or absence of symptoms, the risk of arrhythmias, and the individual preferences of the patient.

Main treatment options include:

1. Implantable cardioverter-defibrillator (ICD).

The only proven treatment for Brugada syndrome. ICD is a device that monitors the heart rhythm and delivers an electric shock to restore normal rhythm if needed. It can be life-saving for people with Brugada syndrome who have had previous episodes of ventricular fibrillation or who are at high risk of developing it. An ICD can prevent sudden cardiac death, but it does not cure the underlying condition.

2. Medication.

Some drugs may help prevent or treat arrhythmias in Brugada syndrome, such as quinidine, isoproterenol, or beta-blockers. However, their efficacy and safety are not well established and they are not a substitute for an ICD.

3. Catheter ablation.

This option is suitable for people who have recurrent arrhythmias despite having an ICD or taking antiarrhythmic drugs. This involves inserting a thin tube (catheter) through a vein in the groin and guiding it to the heart.

The catheter delivers radiofrequency energy to make tiny scars in the heart tissue that trigger the arrhythmia.

It may reduce the need for ICD shock or antiarrhythmic drugs, but complications such as bleeding, infection, heart damage, or stroke may occur.

4. Lifestyle Changes.

Lifestyle changes such as avoiding fever, dehydration, alcohol, and drugs can trigger or worsen the condition.

How to prevent Brugada syndrome?

There is no way to prevent it if you have inherited the genetic mutation or have developed a new mutation. However, you can take steps to reduce the risk of complications and improve your quality of life, such as:

  • Get regular check-ups and follow-ups with your cardiologist.
  • Having an ICD implanted if recommended by your doctor.
  • Take medications as prescribed by your doctor.
  • Avoid drugs that can block sodium channels or affect the heart rhythm.
  • Avoiding medications or drugs that can cause or worsen ventricular fibrillation.
  • Seeking medical attention immediately if you have a fever, infection, or symptoms of arrhythmia.
  • Informing your family members about your condition and encouraging them to get screened for Brugada syndrome.

The Bottom line

Brugada syndrome is a rare but potentially life-threatening condition that affects the electrical activity of the heart. It can cause abnormal heart rhythms that can lead to fainting or sudden cardiac death.

However, with proper diagnosis and treatment, people with Brugada syndrome can lead normal and productive lives.

If you have any questions or concerns about Brugada syndrome, talk with your doctor or cardiologist.

Leave a Comment

Your email address will not be published. Required fields are marked *

Scroll to Top