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Brugada Syndrome: Causes, Symptoms, Treatment, and Cost

Last Updated: Sep 15, 2024

What is Brugada Syndrome?

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Brugada syndrome is a genetic heart disorder that impairs the heart's electrical circuitry and raises the risk of sudden cardiac death. It is named after the Spanish cardiologists who first described the condition, Pedro and Josep Brugada, in 1992.

People with Brugada syndrome have an abnormal heart rhythm that can cause the lower chambers of the heart, known as the ventricles, to beat dangerously fast. This can result in fainting, seizures, and sudden death, especially during sleep or during physical activity.

Mutations in the genes that regulate the movement of ions into and out of the cardiac cells result in Brugada syndrome. This leads to a disruption in the normal electrical signaling in the heart and can cause the heart to beat in an irregular and potentially life-threatening manner.

Types of Brugada Syndrome

Understanding the different types of Brugada syndrome is essential for accurate diagnosis and appropriate treatment. Here are different subtypes of Brugada syndrome;

Type 1 Brugada Syndrome

Type 1 Brugada syndrome is characterized by a specific ECG pattern known as the 'coved-type' pattern. This pattern is suggestive of an elevated risk of sudden cardiac death and ventricular tachycardia. Type 1 Brugada syndrome is the most severe subtype of the condition and is associated with the highest risk of sudden death.

Type 2 Brugada Syndrome

Type 2 Brugada syndrome is characterized by a different ECG pattern known as the 'saddleback' pattern. This pattern is less severe than the coved-type pattern and is associated with a lower risk of sudden cardiac death. Type 2 Brugada syndrome is still considered a serious condition and requires close monitoring and management.

Type 3 Brugada Syndrome

Type 3 Brugada syndrome is a less common subtype of the condition that is characterized by an ECG pattern that changes over time. The ECG pattern may be normal at times and then change to a coved-type or saddleback pattern during periods of stress or physical activity. This type of Brugada syndrome can be more difficult to diagnose and manage, as the ECG pattern may not be consistent.

What causes Brugada Syndrome?

The causes of Brugada syndrome are important for accurate diagnosis and appropriate treatment. Some of the causes of Brugada syndrome include;

Genetic mutations

Brugada syndrome is caused by mutations in genes that control the flow of ions in and out of heart cells. These mutations can be inherited from a parent or can occur spontaneously.

Sodium channel defects

The mutations that cause Brugada syndrome result in defects in the sodium channels in heart cells. This can lead to an erratic and even fatal heartbeat.

Electrolyte imbalances

Electrolyte imbalances, such as high levels of potassium or sodium, can trigger the symptoms of Brugada syndrome.

Medications

Certain medications, such as antiarrhythmic drugs and antibiotics, can trigger the symptoms of Brugada syndrome in some patients.

Substance abuse

Substance abuse, such as alcohol and cocaine use, can trigger the symptoms of Brugada syndrome in some patients.

What are the symptoms of Brugada Syndrome?

The diagnosis of Brugada syndrome can be challenging because the symptoms can be subtle and may not appear until later in life. Some people with Brugada syndrome may not have any symptoms at all, while others may experience sudden cardiac death.

For a precise diagnosis and the best course of treatment, it's crucial to know the causes of Brugada syndrome. The following are a few of the causes of Brugada syndrome;

  • Sudden cardiac arrest: The most serious symptom of Brugada syndrome is sudden cardiac arrest, which can occur without warning and may be fatal.
  • Ventricular tachycardia: Brugada syndrome can cause episodes of ventricular tachycardia, a potentially life-threatening type of irregular heart rhythm.
  • Palpitations: Brugada syndrome can cause episodes of palpitations, which are feelings of a rapid, fluttering, or pounding heartbeat.
  • Fainting: Brugada syndrome can bring on episodes of fainting, which can be triggered by physical or emotional stress.
  • Chest pain: Chest pain brought on by Brugada syndrome can be mistaken for a heart attack.

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What triggers Brugada syndrome?

While the underlying cause of Brugada syndrome is genetic mutations, there are several factors that can trigger an episode of the condition. Understanding the causes of Brugada syndrome is crucial for treating the disorder and lowering the likelihood of life-threatening consequences;

  • Fever: High body temperatures, such as those caused by fever, can trigger symptoms of Brugada syndrome.
  • Alcohol consumption: Alcohol consumption can trigger symptoms of Brugada syndrome, especially in patients who are dehydrated.
  • Cocaine use: Cocaine use can trigger symptoms of Brugada syndrome, as the drug can disrupt the normal electrical signaling in the heart.
  • Electrolyte imbalances: Electrolyte imbalances, such as high levels of potassium or sodium, can trigger symptoms of Brugada syndrome.
  • Stress: Physical or emotional stress can trigger symptoms of Brugada syndrome in some patients.

Who does Brugada syndrome affect?

Brugada syndrome is most commonly diagnosed in people in their 30s and 40s, although the condition can be diagnosed at any age.

The exact incidence of Brugada syndrome is not well-known, but it is estimated to affect between 1 in 1,000 and 1 in 2,000 people worldwide.

The condition is more common in men than in women, and there is evidence to suggest that Brugada syndrome may be more common in certain ethnic populations, such as Southeast Asian populations.

Is Brugada syndrome dangerous?

Brugada syndrome can indeed be harmful. The normal electrical signaling in the heart is disrupted by Brugada syndrome, which affects the electrical system of the heart muscles. This may raise the risk of sudden cardiac death, which can be fatal and occurs suddenly.

The exact risk of sudden cardiac death in people with Brugada syndrome is not well-known, but it is estimated to be higher than in the general population. In some cases, the risk of sudden cardiac death may be as high as 12% over a 10-year period. However, the risk of sudden cardiac death can be reduced with appropriate treatment, such as implantation of an implantable cardioverter-defibrillator (ICD) or lifestyle changes to manage triggers of the condition.

What are the Risk Factors of Brugada Syndrome?

It has been determined that a number of risk factors play a role in the onset and development of the illness. Some of the risk factors for Brugada syndrome include;

  • Family history: People with a family history of Brugada syndrome are at higher risk of developing the condition themselves.
  • Male gender: Brugada syndrome is more frequently diagnosed in men than in women.
  • Southeast Asian ethnicity: People of Southeast Asian ethnicity are at higher risk of developing Brugada syndrome.
  • Other heart conditions: People with other heart conditions, such as long QT syndrome or dilated cardiomyopathy, are at higher risk of developing Brugada syndrome.
  • Certain medications: Certain medications, such as anti-arrhythmic drugs or certain antidepressants, can increase the risk of Brugada syndrome.

How can you prevent Brugada Syndrome?

As Brugada syndrome is predominantly brought on by genetic abnormalities, prevention can be difficult. There are, however, measures that can be taken to lessen the likelihood of major consequences and effectively manage the illness.

Do

  • Individuals with Brugada syndrome should work closely with their healthcare provider to manage their condition and reduce their risk of serious complications.
  • Patients with Brugada syndrome should take their medications as prescribed by their healthcare provider to manage their condition and reduce their risk of serious complications.
  • Regular exercise can help protect the heart and lower the chance of life-threatening consequences in Brugada syndrome patients.

Don’t

  • Patients with Brugada syndrome shouldn't discontinue taking their prescription medications without first speaking to their doctor.
  • Avoiding recreational drugs and alcohol is also advised because they may trigger an episode of the condition.
  • Smoking should be avoided because it raises the risk of significant consequences for Brugada syndrome patients.

Brugada Syndrome - Diagnosis and Tests

Diagnosing Brugada syndrome can be a complex process as the symptoms of the condition can be similar to those of other heart conditions. Early and accurate diagnosis is important in effectively managing and reducing the risk of serious complications in Brugada syndrome patients. There are several tests and diagnostic procedures that healthcare providers can use to diagnose Brugada syndrome, including;

Electrocardiogram (ECG)

A test to gauge the electrical activity of the heart is called an electrocardiogram (ECG). The ECG can show patterns of abnormal electrical activity in the heart that are characteristic of Brugada syndrome. In some cases, healthcare providers may perform an ECG during an episode of the condition to get a clearer picture of the electrical activity of the heart.

Electrophysiology Study (EPS)

The process known as an electrophysiology study (EPS) measures the electrical activity of the heart using specialized cables and tools. The EPS can help healthcare providers identify specific areas of abnormal electrical activity in the heart and determine the best treatment options for Brugada syndrome.

Genetic Testing

If someone has a genetic mutation linked to Brugada syndrome, genetic testing can be employed to find out. Genetic testing can help healthcare providers confirm a diagnosis of Brugada syndrome and determine if other family members may be at risk of developing the condition.

Drug Challenge Test

A drug challenge test is a procedure that involves administering a medication to the patient to see if it triggers an episode of Brugada syndrome. This test can help healthcare providers determine if a specific medication is causing the condition and what the best treatment options are.

What are the possible complications of Brugada Syndrome?

Several health issues, some of which may be life-threatening, can result from brugada syndrome. Early and accurate diagnosis and effective management of Brugada syndrome is essential to reducing the risk of these complications and ensuring that patients live healthy and active lives. Some of the most common complications of Brugada syndrome include;

Sudden Cardiac Death

Sudden cardiac death is one of the most serious complications of Brugada syndrome. Individuals with Brugada syndrome have a higher risk of experiencing sudden cardiac death, which can occur without warning and without any symptoms. Sudden cardiac death can occur during an episode of the condition or in individuals who have never had symptoms before.

Ventricular Fibrillation

It is a serious heart rhythm disturbance that can occur in individuals with Brugada syndrome. The heart beats wildly during ventricular fibrillation, one of the most frequent consequences of Brugada syndrome. This can lead to sudden cardiac death if not treated quickly.

Ventricular Tachycardia

Ventricular tachycardia is another serious heart rhythm disturbance that can occur in individuals with Brugada syndrome. During ventricular tachycardia, the heart beats too quickly and can cause a loss of consciousness, fainting, or even sudden cardiac death.

Pulseless Electrical Activity (PEA)

The electrical activity of the heart during PEA becomes aberrant, which makes it difficult for the heart to adequately pump blood. This is one of the most frequent consequences of Brugada syndrome. If this is not addressed right away, it may result in abrupt cardiac death.

Home Remedies for Brugada Syndrome?

While there is no cure for Brugada syndrome, there are a number of home remedies and lifestyle changes that can help manage the symptoms and reduce the risk of serious complications. Among these natural therapies are;

  • Maintaining a balanced diet: A balanced diet full of fresh produce, healthy grains, and lean proteins helps promote overall heart health and lower the chance of developing heart disease.
  • Exercising regularly: Regular physical activity can help lower the risk of heart disease and enhance cardiovascular health. It is important to speak with a doctor before starting a new exercise program, especially if you have Brugada syndrome.
  • Avoiding triggers: Triggers such as alcohol, caffeine, and certain medications can worsen the symptoms of Brugada syndrome. To lower the chance of life-threatening problems, it is crucial to recognize and stay away from certain triggers.
  • Managing stress: Stress can increase the signs of Brugada and have a detrimental effect on heart health. Techniques such as meditation, yoga, and deep breathing can help manage stress and improve overall heart health.
  • Staying hydrated: Staying hydrated by drinking plenty of water and other fluids can help support overall heart health and reduce the risk of heart disease.
  • Taking prescribed medications: Taking prescribed medications as directed can help manage the symptoms of Brugada syndrome and reduce the risk of serious complications. Before altering your pharmaceutical prescription, it is crucial to consult a doctor.

What to eat in Brugada Syndrome?

Diet plays a crucial role in managing Brugada syndrome and maintaining overall heart health. A nutritious diet can enhance overall cardiovascular health and lower the risk of heart disease. When it comes to managing Brugada syndrome, it is important to focus on the following dietary guidelines:

  • Fruits and vegetables are rich in essential vitamins and minerals, as well as antioxidants and fiber, that can help support overall heart health.
  • Whole grains, such as whole wheat, brown rice, and oatmeal, are a good source of fiber, vitamins, and minerals that can help support overall heart health.
  • Lean protein sources, such as chicken, fish, and legumes, are important for maintaining overall heart health and reducing the risk of heart disease.
  • Processed and high-fat foods, such as fried foods and fast food, can increase the risk of heart disease and worsen the symptoms of Brugada syndrome.
  • High salt intake can raise blood pressure and exacerbate Brugada syndrome symptoms. It's crucial to keep salt intake in check and, whenever possible, choose low-sodium products.
  • Alcohol use can aggravate Brugada syndrome symptoms and raise the danger of life-threatening consequences. It's crucial to either moderate or completely prevent alcohol usage.

What not to eat in Brugada Syndrome?

When it comes to managing Brugada syndrome, it is just as important to be mindful of what not to eat as it is to focus on a healthy diet. The following foods should be limited or avoided altogether;

  • Processed foods: Processed foods are often high in salt, sugar, and unhealthy fats, all of which can worsen the symptoms of Brugada syndrome and increase the risk of heart disease.
  • High-fat foods: The risk of heart disease might rise while eating foods high in harmful fats including trans and saturated fats.
  • Salt-rich foods: Foods that are high in salt can increase blood pressure and worsen the symptoms of Brugada syndrome.
  • Alcohol: Alcohol use can aggravate Brugada syndrome symptoms and raise the danger of life-threatening consequences.
  • Caffeine: Caffeine can trigger arrhythmias in some patients with Brugada syndrome and should be limited.

Some fruits and vegetables, such as tomatoes and grapefruit, contain compounds that can interact with certain medications used to treat Brugada syndrome and should be avoided.

Brugada Syndrome Treatments

Brugada syndrome is a serious heart condition that requires careful management to prevent life-threatening complications. Brugada syndrome does not yet have a cure, however there are a number of treatments that can be used to control the illness and lower the chance of unexpected death. These treatments include;

Medications

Brugada syndrome is a serious heart condition that requires careful management to prevent life-threatening complications. Brugada syndrome does not yet have a cure, however there are a number of treatments that can be used to control the illness and lower the chance of unexpected death. These treatments include;

Medications

Medications can help regulate heart rhythm and prevent dangerous arrhythmias in people with Brugada syndrome. Here are few medicines that are often given to manage the symptoms of Brugada syndrome in patients;

Sodium channel blockers

These medications are used to slow down the electrical signals in the heart and prevent arrhythmias.

Antiarrhythmic drugs

Antiarrhythmic drugs can help regulate heart rhythm and prevent dangerous arrhythmias.

Beta blockers

Beta blockers can help slow down heart rate and avoid arrhythmias.Implantable Cardiac Devices

Health experts also use Implantable cardiac devices to help monitor heart rhythm and deliver therapeutic shocks if necessary. Some of the devices are;

Implantable cardioverter-defibrillator (ICD)

An ICD is a tiny device that tracks heartbeat rhythm and is placed under the skin. The ICD administers an electrical shock to correct a hazardous arrhythmia and return the heart to normal rhythm.

Pacemaker

A pacemaker is a tiny gadget that is inserted beneath the skin to help control heartbeat.

Catheter Ablation

Catheter ablation is a minimally invasive surgery that uses severe heat or cold to kill the cardiac tissue responsible for arrhythmias.Surgery

In severe cases of Brugada syndrome, surgical procedures are used to prevent arrhythmias and improve overall heart health.

Maze procedure

The Maze procedure is a surgical procedure used to treat various heart conditions, including Brugada syndrome. It involves creating a series of incisions in the heart muscle to create a pattern, or 'maze,' of scar tissue. This scar tissue helps prevent the electrical signals in the heart from short-circuiting, which can cause dangerous arrhythmias. The operation is often carried out by combining minimally invasive methods with conventional open-heart surgery.

Formation of a surgical scar

The formation of a surgical scar is another surgical treatment option for Brugada syndrome. This procedure involves intentionally creating a scar on the heart muscle, which can help prevent arrhythmias. The surgical scar acts as a physical barrier that blocks the electrical signals from short-circuiting and causing dangerous arrhythmias.

This procedure is typically performed using minimally invasive techniques, such as catheter ablation, and is often used in conjunction with other treatments, such as medication and implantable cardiac devices, to effectively manage Brugada syndrome.

Which doctor to consult for Brugada Syndrome?

If you suspect you have Brugada syndrome, it is important to consult with a cardiologist, a specialist in diagnosing and treating heart conditions. A cardiologist will be able to perform a thorough evaluation, including a medical history, physical examination, and various diagnostic tests, to determine if you have Brugada syndrome and what the best course of treatment is for you.

Your unique demands and medical background will be taken into consideration as they collaborate with you to create a customized treatment plan. After receiving a Brugada syndrome diagnosis, it is crucial to follow up with your cardiologist frequently to monitor your health and make any required changes to your treatment regimen.

Which are the best medicines for Brugada Syndrome?

There are several medications that are commonly used to treat Brugada syndrome and regulate heart rhythm, including:

Sodium channel blockers: These medications help slow down the electrical signals in the heart, reducing the risk of arrhythmias. Examples include;

Beta-blockers: Beta-blockers can help regulate heart rate and prevent dangerous arrhythmias in people with Brugada syndrome. Examples include;

Anti-arrhythmic agents: These medications are used to prevent dangerous arrhythmias from occurring. Examples include;

  • Amiodarone
  • Sotalol

Calcium channel blockers: Calcium channel blockers can help regulate heart rhythm and prevent arrhythmias in people with Brugada syndrome. Example include;

  • Verapamil
  • How long does it take to recover from Brugada Syndrome?

    The length of time it takes to recover from Brugada syndrome can vary greatly depending on a number of factors, such as the underlying cause of the condition, the severity of symptoms, and the type of treatment received.

    For some individuals, the symptoms of Brugada syndrome can resolve on their own or with the use of medication. For others, the condition may require more aggressive treatment, such as implantable cardiac devices or surgery, and the recovery period may be longer.

    In general, recovery time can range from several weeks to several months, and it is important to work closely with your healthcare provider to develop a personalized recovery plan that takes into account your individual needs and medical history.

    Are the results of the treatment permanent?

    The results of treatment for Brugada syndrome can vary, and it is not always possible to determine the long-term outcome. For some individuals, the results of treatment may be permanent, while for others, the symptoms may return or worsen over time.

    Implantable cardiac devices, such as defibrillators, can be highly effective in preventing life-threatening arrhythmias and improving quality of life, but the devices may need to be replaced or reprogrammed over time.

    Surgery can also be effective in treating Brugada syndrome, but it may not always provide a permanent solution. In some cases, the underlying cause of the condition may return or progress, leading to the return of symptoms.

    Who is eligible for the treatment?

    Individuals who have been diagnosed with Brugada syndrome are eligible for treatment. This condition is often diagnosed in people who have a family history of sudden cardiac death, but it can also occur in those without any known family history. Treatment options will vary depending on the severity of the condition and the individual's age, overall health, and personal preferences.

    Who is not eligible for the treatment?

    There is no one who is completely ineligible for treatment of Brugada syndrome. However, some treatments may not be appropriate or effective for certain individuals, such as those with severe or advanced heart disease or other underlying medical conditions. In these cases, the best course of action may be to manage the condition through lifestyle modifications and close monitoring.

    What are the post-treatment guidelines?

    After being treated for Brugada syndrome, it is important to follow certain guidelines to ensure optimal health and reduce the risk of future complications. These guidelines may vary depending on the specific treatment received, but may include the following;

    • It is important to regularly check in with your doctor to monitor your condition and assess the effectiveness of your treatment plan
    • If prescribed medication, it is important to take it as directed and report any adverse effects to your doctor
    • Maintaining a healthy lifestyle, including eating a balanced diet, getting regular exercise, and managing stress, is essential for optimal health and can help reduce the risk of future complications
    • Certain triggers, such as alcohol, drugs, and sleep deprivation, can increase the risk of arrhythmia in individuals with Brugada syndrome. It's crucial to keep these triggers as far away as possible
    • It is important to be aware of symptoms, such as chest pain or irregular heartbeat, and seek prompt medical attention if they occur.

    What is the price of Brugada Syndrome treatments in India?

    Depending on the kind of treatment and equipment employed, Brugada Syndrome treatment prices might vary significantly in India. Implantable Cardiac Devices (ICD) are known to be extremely expensive and the cost of implantation in India can range anywhere from 2 to 20 lakh rupees.

    On the other hand, the cost of Quinidine tablets, which are commonly used to treat Brugada Syndrome, is around 56 rupees for 10 tablets. It is important to note that these prices may change with time and can vary depending on the location, the doctor, and the hospital where the treatment is performed.

    Additionally, insurance coverage and medical financing options can also greatly impact the overall cost of treatment for Brugada Syndrome.

    What are side-effects of Brugada Syndrome treatments?

    Treatments for Brugada Syndrome, like any medical procedure, may have negative effects of their own. It is important to be aware of these potential side effects in order to understand what to expect from treatment and make informed decisions.

    The severity and type of side effects can vary greatly depending on the specific treatment and individual patient, but some common side effects include;

    • Dizziness and fainting
    • Nausea and vomiting
    • Drowsiness or fatigue
    • Skin irritation or infection at the site of an ICD implant
    • Alterations in heartbeat or rhythm
    • Aching or weakened muscles
    • Allergic reactions to medications

    Brugada Syndrome - Outlook / Prognosis

    Brugada Syndrome is a rare inherited cardiac condition that affects the heart's electrical system, potentially leading to dangerous heart rhythms and sudden cardiac death. The prognosis for individuals with Brugada Syndrome depends on various factors, including the type of genetic mutation causing the syndrome, the presence of symptoms, and the individual's response to treatment.

    In general, individuals with Brugada Syndrome who have never experienced symptoms such as fainting, seizures, or cardiac arrest have a relatively good prognosis. However, those who have experienced symptoms, especially cardiac arrest, are at a higher risk of future events and require close monitoring and aggressive treatment.

    Treatment options for Brugada Syndrome include implantable cardioverter-defibrillators (ICDs), which can detect and correct dangerous heart rhythms, and medications such as quinidine, which can help prevent arrhythmias. In some cases, ablation therapy, a procedure that destroys small areas of heart tissue responsible for abnormal electrical activity, may also be an option.

    It is important for individuals with Brugada Syndrome and their family members to undergo genetic testing and receive appropriate medical management to minimize the risk of sudden cardiac death. With appropriate treatment and close monitoring, many individuals with Brugada Syndrome are able to lead normal, healthy lives.

    References

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    Written ByDr. Rahul Gupta MD - Internal Medicine,MBBS,DM - Cardiology,Fellow European Society of CardiologyCardiology
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