What Is Brugada Syndrome?
Brugada syndrome (BrS) is a rare genetic heart condition. Sodium channel gene mutations can change how your heart responds to electric signals. And you may get ventricular tachycardia (V-tach), a life-threatening abnormal heart rhythm in the lower chambers of your heart.
BrS causes about 12 out of every 100 sudden cardiac deaths in structurally healthy hearts. But many people don't get symptoms. And with Brugada syndrome treatment, you can live a normal life span.
About 3 to 5 in 10,000 people in the world have Brugada syndrome. But only 0.01% have the syndrome in the West. It's more common in people of Japanese and South Asian descent.
Is Brugada syndrome a hereditary heart condition?
Yes, Brugada syndrome is a hereditary heart condition caused by changes in sodium channel genes. The syndrome is usually passed down from one parent (autosomal dominant).
Many different genes contribute to the syndrome. But only the SCN5A and SCN10A genes have been directly linked to Brugada syndrome.
What Are the Symptoms of Brugada Syndrome?
You may not have any symptoms with Brugada syndrome. Almost 60 in 100 people with Brugada don't have signs when they're first diagnosed.
Here are some common Brugada symptoms. Depending on your symptoms, you may feel:
Arrhythmia. Your heart can beat too fast. You may feel like your heart is fluttering, skipping a beat, or pounding in your chest. You can have pain, get short of breath, or feel dizzy.
Palpitations. Your upper heart chambers can flutter and beat too quickly (atrial fibrillation). You may feel like your heartbeat is doubling, racing, or flip-flopping.
Pass out (syncope). Because your lower heart chambers beat too fast, you can faint without warning. You can feel lightheaded, nauseated, or short of breath.
Bradycardia. Your heartbeat can slow down. You may feel chest pain, get confused, or feel lightheaded. You can also get tired, feel faint, or get short of breath.
Ventricular tachycardia. This is also called polymorphic ventricular tachycardia (V-tach). In this, your heart rate can get over 100 beats per minute. Your heart chambers can't fill with blood. You may get lightheaded or pass out. If V-tach lasts too long, it can be life-threatening.
Ventricular fibrillation. Your lower heart chambers can twitch, beating abnormally. Ventricular fibrillation (VFib) can also be life-threatening. You can fall down or pass out — often without warning. You may have chest pain or dizziness or feel nauseated. You can feel your pulse race or have trouble breathing.
Cardiac arrest. With this serious life-threatening condition, your heart can get damaged or stop. You can have chest pain, shortness of breath, or weakness or get heart palpitations.
Sudden unexpected nocturnal death syndrome (SUNDS). About 4%-12% of sudden cardiac deaths are from BrS. Often, the first sign is gasping for air at night (nocturnal agonal respiration). This nightly symptom usually comes before sudden unexpected nocturnal death syndrome.
Depending on where you live, SUNDS is also called:
- Sudden unexpected death syndrome (SUDS)
- Lai tai (death while asleep) in Thailand
- Bangungut (nightmare followed by death) in the Philippines
- Pokkuri (sudden unexpected death during the night) in Japan
You can also get extra heartbeats (premature ventricular beats). These extra beats can sometimes cause VFib. The symptoms commonly happen at night during your sleep. Learning about your symptoms can help you avoid triggers for abnormal heart rhythms.
What Causes Brugada Syndrome?
Changes in sodium channel genes most commonly cause Brugada. This means your heart doesn't respond to electrical signals normally. Your heart can beat rapidly or get an unusual heart rhythm.
Here are some common genes linked to Brugada syndrome:
- Sodium channel SCN5A gene mutations cause about 15 to 30 out of 100 Brugada cases. It can raise your risk for heart problems at a younger age.
- Sodium channel SCN10A gene changes are only found in 17 out of 100 Brugada cases. These gene changes can raise your risk for V-tach and sudden cardiac death.
- Other sodium channel genes such as SCN1B or GPD1-L
- Calcium channel genes such as the cardiac L-type calcium channel
- Potassium channel KCNE3 and KCNE2 genes
- Unknown genes on the small arm of chromosome 3 (3p22-25)
But you may not have symptoms. The gene changes may only cause the Brugada pattern — specific changes on your electrocardiogram (ECG or EKG).
What triggers Brugada?
Medical conditions and medications can trigger symptoms such as:
Fever. A higher body temperature may cause Brugada symptoms.
Medications. Some medicines can unmask abnormal heart rhythms. Sodium channel blockers are usually used to diagnose Brugada syndrome. Some trigger medications to avoid include beta-blockers and tricyclic and tetracyclic antidepressants.
BrugadaDrugs.org can help you avoid problematic medicines.
Substance use. Avoid alcohol and cocaine — they can trigger Brugada symptoms. It's also best to avoid other drugs and smoking.
Problems with metabolism. Certain metabolic problems, such as unbalanced electrolytes, can cause irregular heartbeats. Some studies suggest high carbohydrate meals before bed can lower potassium, and lower potassium can lead to heart attack during the night with Brugada syndrome.
Autonomic tone. Your autonomic tone (nervous system balance) can affect your heart rate. If your nerves gets unbalanced, you can get a faster heart rate during the night.
How Is Brugada Syndrome Diagnosed?
Brugada syndrome diagnosis combines your medical history, EKG pattern, and clinical symptoms. Once your cardiologist (specializes in the heart) suspects Brugada syndrome, they'll examine you and order tests. They'll also check your heart to make sure it's structurally normal.
Your doctor may order an EKG or cardiac MRI or both. This can rule out other reasons for V-tach. You might also get a cardiac stress test to make sure you don't have coronary artery problems.
Electrocardiogram for Brugada syndrome
The 12-lead EKG test helps diagnose Brugada syndrome. The test checks for any abnormal heart patterns, called a Brugada pattern. And the Brugada pattern is about nine times more common in men.
Special electrodes are placed on your chest to measure electric stimuli as your heartbeats. The EKG uses electricity to graph the voltage of your heart rhythm over time.
Your doctor will check for ST and T wave changes. That's the time before your upper heart chamber (atrium) contracts. The QRS wave may also change. This wave measures your lower heart chamber contraction (ventricle).
There are two types of EKG Brugada patterns:
Type 1 Brugada pattern. If you have a type 1 pattern, your EKG may have:
- A higher, coved, or concave-shaped ST wave (at least 2 millimeters)
- T wave that goes downward (negative)
- Wider QRS wave (incomplete or complete right bundle branch block)
Type 2 Brugada pattern. If you have a type 2 pattern, your EKG may show:
- A higher ST wave that looks like a saddleback (at least 2 millimeters)
- Two-phased (positive) T wave
It can be hard to tell if an ECG pattern means you have the syndrome. Sometimes, blocking sodium channels in your heart helps diagnose Brugada syndrome.
Medication challenge
Success for the challenge ranges from 15% to 100%. Your doctor may set up a medication challenge if you have a Brugada type 1 pattern and you don't have a history of Brugada or heart attack. You'll also get the challenge with a type 1 pattern if you haven't fainted or had V-tach problems.
You'll get the challenge test with Brugada type 2 pattern if you have symptoms. But without symptoms, your doctor will likely do the test if you have a type 1 family history or heart attack before the age of 45.
Because there's some risks, your doctor will monitor you and stop testing if you get heart symptoms.
Electrophysiology studies (EPS)
Most people don't need an EPS. But it can help your doctor find your arrhythmia. Once your doctor knows where your abnormal heartbeat is, they can sometimes fix it.
Cardiac monitor
Your cardiologist may also put a cardiac monitor under your skin, called an insertable cardiac monitor (ICM). The small device reports your abnormal heartbeat back to you. If you're at higher risk for heart attack, an ICM can lower your chances for fainting and palpitations.
Genetic test
Your doctor may do a blood test to check for changes in sodium channel genes (SCN5A and SCN10A). Remember, genetic testing is limited. A negative test doesn't rule out Brugada syndrome. And a positive test doesn't mean you'll get symptoms either.
Brugada Syndrome Treatment
If you have BrS, your doctor may suggest ways to prevent arrhythmia. They may prescribe medicine to lower your fever. Regular checkups also help review your health and medications and update your treatment plan. Your doctor can also check for any new heart problems.
Implantable cardioverter-defibrillator
If you're at higher risk, you may get an implantable cardioverter-defibrillator (ICD). It's like an ICM but can also send a shock to restart your heart, if needed. The ICD goes under your skin to monitor and correct your heartbeat.
The device checks your heart's rhythm during your sleep, too. If you get an unusual heartbeat, the shock can safely reset your heart rhythm. An ICD can also lower your risk for sudden cardiac death.
Other options to treat Brugada
If an ICD isn't an option, your doctor may suggest:
- A procedure to destroy the areas in your heart causing the abnormal rhythm (catheter ablation)
- Quinidine to prevent abnormal heart rhythms
- Amiodarone to lower your risk for an abnormal heartbeat
Living With Brugada Syndrome
You may have many different feelings about living with a chronic, high-risk condition. You can help lower your risk and prepare to take action yourself. You and your doctor can talk about the best treatments.
An automatic external defibrillator (AED) at your home or your child's school can ease your mind. Also, ask people who live or work with you to learn CPR.
There are also many lifestyle changes to help with BrS. Here are some ways to lower your risk for an abnormal heart rhythm:
- Treat any fever with medication (acetaminophen or ibuprofen).
- Stay hydrated and drink lots of fluids.
- Avoid getting overheated (heat exhaustion).
- Talk to your doctor about taking omega-3 fatty acid (fish oil supplement).
- Avoid eating at least two to three hours before bedtime, especially foods high in carbohydrates.
Certain medications, alcohol, and substances can make symptoms worse. Avoid smoking, alcohol, and drugs. You're also at risk for dehydration if you exercise hard, have diarrhea, or vomit.
You can try drinking an oral rehydration solution (ORS) if you get sick. And you may want to avoid hard exercise or sports if you're at high risk. Talk to your doctor about the best physical exercise for your health.
What foods should I avoid with Brugada Syndrome?
It's still unclear, but some foods may trigger an abnormal heartbeat. Some studies suggest avoiding or limiting:
- Alcohol
- Energy drinks
- Flavonoids, an antioxidant found in foods such as grapefruit, onions, and chocolate
- Licorice
- Honey
When should you see a doctor?
Have a follow-up at least yearly. If you have an ICD, check your device at least every six months. Tell your doctor about any unusual readings.
If you're at higher risk, think about letting your coworkers know about your medical condition. Telling others about your risk gives them a chance to help you.
Get medical help right away if you have:
- Pain in your chest
- An irregular, pounding heartbeat
- A racing heartbeat
- A hard time breathing or wheezing without reason
- Fainting or passing out
- Dizziness or getting lightheaded
- A seizure
Brugada Syndrome FAQs
Is Brugada a terminal illness?
Brugada syndrome is not always terminal. With medical treatment, most people with the syndrome live normal lives.
Although the syndrome can be life-threatening, most people with BrS don't have symptoms. In fact, more than 70 out of every 100 people with Brugada syndrome don't have symptoms.
What's the life expectancy of someone with Brugada syndrome?
Your life expectancy can vary depending on your gene changes, medical conditions, and other life factors. Although the average age for sudden cardiac death is 35-40, with treatment, most people with Brugada syndrome live normal life spans.
At what age does Brugada syndrome start?
Brugada syndrome starts at birth because it's genetic; it's in your DNA. But Brugada symptoms typically show up around 40 years of age. Remember, most people with the syndrome don't get symptoms. And Brugada pattern is about nine times more common in men.
Can you exercise with Brugada?
If you're low risk or you don't have symptoms, low intensity exercise is likely OK. But with Brugada syndrome, it's better to always talk to your doctor about the best exercise for your health. Although symptoms often happen at night, most doctors suggest avoiding harder activities.
Can COVID-19 cause Brugada?
The COVID-19 virus doesn't directly cause Brugada syndrome. But a high fever can trigger BrS. Because the virus can cause a high fever, a higher temperature can cause Brugada symptoms.
If you get a fever and have Brugada syndrome, ask your doctor about taking medicine to lower it. Keep your body temperature down to lower your chances of unusual heart rhythms.