Is your heart suddenly beating strangely? Could it be Brugada Syndrome?

Have you ever suddenly lost consciousness, felt dizzy? Or had a feeling of rapid heartbeat, or difficulty breathing? Sometimes these can be caused by something not serious. However, in rare cases, they can be symptoms of a heart condition called Brugada Syndrome. Let's talk about this a little bit today, because it's important to be aware of it.

What is Brugada Syndrome?

Simply put, Brugada Syndrome is a condition that causes abnormal electrical activity in the lower chambers of your heart, called the ventricles. This can cause your heart to suddenly lose its rhythm. This is called ventricular fibrillation or v-fib . Imagine your heart fluttering instead of pumping blood properly. This causes your brain to not get enough blood. That's when you lose consciousness (syncope) .

This can be a dangerous condition, because this `v-fib` can even lead to sudden cardiac death . Most of the time, this happens when you are at rest or sleeping. Researchers say that Brugada Syndrome is responsible for about 4% of sudden cardiac deaths worldwide.

But this is not a very common disease. About three to four people out of ten thousand have this condition.

What symptoms appear?

Brugada Syndrome symptoms can appear at any age. However, they usually begin around the age of 40. Some people may not show any symptoms at all. More than 70% of people are said to have no symptoms. However, if symptoms do appear, they may include:

• Abnormal heartbeat (`ventricular arrhythmia`) : This means that the heart's rhythm is disturbed.
• Syncope: Sudden loss of consciousness and collapse.
• Dizziness : A feeling of spinning.
• Difficulty breathing .
• Heart palpitations : A feeling of fluttering or spinning in the chest.
• Cardiac arrest : This is sometimes the first symptom to appear. Some people may die in their sleep because of this.

Important: If you experience one or more of these symptoms frequently, it is best to see a doctor for advice.

What are the triggers for Brugada syndrome?

Certain things can cause Brugada syndrome symptoms to appear suddenly or worsen. We call these `triggers`.

• Fever : These symptoms can occur when a person with Brugada syndrome develops a fever. Therefore, it is very important to reduce the fever quickly if the person has a fever. Even if you have an implantable cardioverter defibrillator (ICD), you still need to reduce the fever. This can be done with over-the-counter fever-reducing medications.
• Heat exhaustion / Dehydration : Water retention in the body, exhaustion due to extreme heat.
• Some medications :
• Sodium channel blockers.
• Lithium.
• Tricyclic antidepressants.
• Alcohol : Drinking too much alcohol.
• Drugs : Using drugs like cocaine and marijuana.

It is very important to be aware of these things and avoid them as much as possible.

What are the reasons for this?

The exact cause of Brugada Syndrome is sometimes unknown . Or it may be genetic . It is still not known exactly which genetic mutation causes this disease in about 70% of people.

However, some people have 18 or more genetic variations. The most common of these is a variation in a gene called SCN5A . These variations disrupt the way electrical signals in the heart travel.

This is a disease that can be inherited from just one parent . That is, a child of someone with a gene mutation associated with Brugada syndrome has a 50% chance of developing it.

Who is more likely to develop this? (Risk Factors)

This disease is more common in men . It is about 8 to 10 times more common in men than in women.

Also, if someone in your family has died from sudden cardiac arrest, or has been diagnosed with Brugada syndrome , it would be wise for you to get tested for this as well.

Another thing is that it is said that this disease is slightly more common among people of Asian descent .

How does a doctor diagnose this? (Diagnosis)

To determine if you have Brugada Syndrome, a doctor will do the following:

• You will be given a physical exam .
• Your medical history will be reviewed.
• They will ask about your family's health history (biological family history) , especially to see if anyone in your family has had a sudden cardiac death.
• They will ask you to do several special tests .

What tests are performed?

There are several main tests used to diagnose this disease:

• Genetic testing : This test can take a sample of your saliva or blood and determine if you have a specific gene mutation associated with Brugada syndrome. It is also a good idea to get this test done to see if other people in your family also have this gene mutation. It is especially important to get screened for this if you have a close relative (`first-degree relative`) with Brugada syndrome, such as your parents, siblings, or children.
• Electrocardiogram (EKG or ECG) : This is used to record the electrical activity of your heart as it beats. Doctors look for a specific pattern (the Brugada syndrome type 1 EKG pattern) on the EKG. Sometimes a weak pattern called type 2 or type 3 can be seen, but that alone cannot confirm the disease. However, due to the aforementioned triggers (e.g. fever), this type 2 or type 3 pattern can change to a type 1 pattern. Sometimes this pattern is not immediately visible on the EKG, so the doctor may repeat the EKG two or three times, changing the leads. Sometimes, an EKG is also performed after a full meal.
• EKG with medication (drug challenge test) : Before the EKG, your doctor may give you a medication that will help your EKG show up more clearly if you have Brugada syndrome. This test is used to see if your EKG has a type 2 or type 3 pattern, which can change to a type 1 pattern.

Depending on your EKG results, further tests may be done:

• Electrophysiology (EP) testing : This involves passing a thin tube (catheter) through a large vein in your leg (femoral vein) into your heart to measure the electrical activity inside your heart. This may be done if the diagnosis is not clear after other tests.
• Lab tests: Blood tests are done to check the levels of electrolytes such as potassium, calcium, and magnesium in the blood, and to check for other possible causes of irregular heart rhythms.

How to treat?

The main goal of treating Brugada Syndrome is to prevent the occurrence of ventricular arrhythmias and to quickly control them if they occur.

You can give yourself treatments like this:

• Medications like Isoproterenol .
• Medicines like quinidine .
• An implantable cardioverter defibrillator (ICD): This device can be implanted if you have had a heart attack, such as ventricular tachycardia, fainting, or cardiac arrest. It detects a dangerous heart rhythm and delivers a small electrical shock to the heart to restore it to normal.
• Ablation ( in some cases): This involves identifying and destroying the areas in the heart that are causing abnormal electrical signals.

Even if you don't have symptoms, your doctor may decide to insert an ICD based on your family history and test results. Some doctors may decide to check you regularly and only treat you if you develop symptoms. However, other doctors may not agree with this approach, as your first symptom may be sudden cardiac death.

Are there any complications in the treatment?

ICDs are not 100% foolproof devices. Sometimes things like this can happen:

• You can give a shock at the wrong time .
• There may be a fault in one of the leads in the machine.
• An infection may occur around the machine.

Therefore, after having an ICD implanted, it is very important to go for tests exactly as the doctor recommends.

How long does it take to heal?

After an ICD is inserted, you can do most of your normal activities within a few days. However, you will need to stop driving for about a week. You can do some strenuous work every day, but don't do it to the point of exhaustion. Don't lift weights or do strenuous exercise until your doctor tells you to.

Can this be prevented? (Prevention)

If you inherit Brugada Syndrome from your parents, it cannot be changed. However, if you know that someone in your family has Brugada Syndrome, you and your relatives can have a genetic test to see if you have it.

Also, if you are hoping to have a child, you can meet with a genetic counselor and talk to them about the risk of passing this on to your children.

What can you expect when living with Brugada syndrome?

There is no cure for Brugada Syndrome. However, treatment can greatly reduce the risk of sudden cardiac death.

People with Brugada syndrome who have symptoms but are not receiving treatment are at increased risk of sudden cardiac death. This risk is much lower in people who are asymptomatic and have a normal EKG.

How do I take care of myself? (Self-care)

If a doctor tells you that you have Brugada Syndrome, the most important thing is to avoid as many of the triggers that can disrupt that rhythm as possible.

• Prevent fever . If you develop a fever, bring it down quickly.
• Stay away from certain medications and substances . Ask your doctor for a list of medications that you are allergic to.
• Avoid excessive alcohol and drugs completely .
• Drink plenty of water to avoid dehydration .
• Be careful when doing strenuous activities .

When do you need to see a doctor? / Do you need to go to the emergency room?

If you have Brugada syndrome, you should see your doctor at least once a year for a check-up . If you have an ICD, you should have it checked at least twice a year . If you feel anything unusual, tell your doctor .

Emergency! If you have a cardiac arrest, you will not be able to help yourself. Therefore, you will need someone to help you. If you are at risk of cardiac arrest, tell your family to learn CPR (Cardiopulmonary Resuscitation) , and call 1990 (Sri Lanka's emergency ambulance service) or the nearest emergency number. Also inform your workplace about this risk.

Important questions to ask your doctor

When you go to see the doctor, don't forget to ask questions like these:

• Do I need to have an ICD?
• How should an ICD be maintained?
• Can you tell me about a support group where other people with this condition can come together?
• What medications should I avoid? Can you give me a list?
• Should the rest of my family be tested for this?

Take-Home Message

It's normal to feel overwhelmed when you learn that you have a condition that could lead to a sudden heart attack. However, knowing that you are at risk gives you the opportunity to take steps to reduce that risk . Your doctor will find the best treatment option for you.

If possible, it would be a great relief if your home and your child's school had an Automatic External Defibrillator (AED) . Also, ask your neighbors and coworkers to learn CPR. These things can help save lives. Always follow your doctor's instructions, get regular checkups, and avoid life-threatening triggers .

` Brugada syndrome, cardiac arrhythmias, sudden cardiac arrest, genetic diseases, EKG, ICD, heart disease