Brugada Syndrome
An inherited electrical disorder of the heart
Quick Facts
- Type: Inherited heart rhythm disorder
- Main risk: Dangerous arrhythmias and cardiac arrest
- Often diagnosed in: Young to middle-aged adults
- Key treatment: Implantable defibrillator in high-risk cases
Overview
Brugada syndrome is a rare inherited condition that affects the heart's electrical system, the network that coordinates each heartbeat. In people with this syndrome, faulty electrical signaling can occasionally trigger a very fast, disorganized heart rhythm. If this rhythm does not stop on its own, it can prevent the heart from pumping blood effectively and lead to fainting or sudden cardiac arrest.
The condition is often present from birth but may not cause problems until adulthood, and some people never have symptoms. It is most commonly identified in young to middle-aged adults and tends to run in families. Because the first sign can be a life-threatening event, recognizing and managing the condition is important, especially when there is a family history.
Symptoms
Many people with Brugada syndrome have no symptoms and are diagnosed because of a family history or an abnormal heart tracing found by chance. When symptoms occur, they may include:
- Fainting or near-fainting, often without warning
- Palpitations or a sensation of a racing, pounding heart
- Episodes that happen during rest or sleep, or with fever
- Seizure-like activity from lack of blood flow to the brain
- Sudden cardiac arrest, which can be the first sign
Symptoms are more likely to appear at night, with fever, or when triggered by certain medications.
Causes
Brugada syndrome is usually caused by inherited changes in genes that control the flow of electrical ions, especially sodium, across heart cell membranes. The most commonly involved gene is one that affects sodium channels in the heart.
- Genetics: The condition is often passed down in families, and a parent with the gene has a chance of passing it to each child.
- Triggers: Fever, certain medications, excessive alcohol, and electrolyte imbalances can provoke dangerous rhythms in people who carry the trait.
In some cases the specific genetic change is not identified even when the syndrome is present.
Risk Factors
- A family history of Brugada syndrome or unexplained sudden death
- Being male
- Asian ancestry, in which it appears more commonly
- A previous episode of fainting or cardiac arrest
- Fevers and certain medications that can unmask the condition
Diagnosis
Diagnosis centers on the heart's electrical tracing and family history:
- Electrocardiogram (ECG): Looks for a characteristic pattern; sometimes a medication is given under close monitoring to bring out the pattern.
- Genetic testing: Can identify a known gene change and help screen relatives.
- Family screening: Evaluating close relatives, since the condition is inherited.
- Electrophysiology study: Specialized heart-rhythm testing in selected cases.
Treatment
Treatment is tailored to how high a person's risk is:
- Implantable cardioverter-defibrillator (ICD): The main treatment for high-risk people; this device detects a dangerous rhythm and delivers a shock to restore a normal beat.
- Avoiding triggers: Promptly treating fevers, avoiding certain medications, and limiting alcohol.
- Medication: Drugs such as quinidine may help in some people to reduce dangerous rhythms.
- Monitoring: Regular follow-up with a heart specialist, especially for those without an ICD.
People at lower risk may not need a device but should still avoid triggers and stay under specialist care.
Prevention
- Treat fevers promptly with fever-reducing medication
- Check with a doctor or pharmacist before taking new medications, as some can be risky
- Limit alcohol and avoid recreational stimulant drugs
- Make sure close relatives are screened if you are diagnosed
- Follow your cardiologist's monitoring and treatment plan
When to See a Doctor
See a heart specialist if you have unexplained fainting, palpitations, or a family history of Brugada syndrome or sudden unexplained death, especially at a young age. Call emergency services immediately if someone collapses, is unresponsive, or is not breathing normally; begin CPR and use an automated external defibrillator if available, as sudden cardiac arrest needs treatment within minutes.
Frequently Asked Questions
What is Brugada syndrome?
Brugada syndrome is a rare inherited disorder of the heart's electrical system that can cause sudden, dangerously fast and chaotic heartbeats. These abnormal rhythms can lead to fainting or sudden cardiac arrest, sometimes in otherwise healthy people.
Is Brugada syndrome inherited?
Yes, it is usually genetic and can be passed from a parent to a child. Because of this, when one person is diagnosed, doctors often recommend screening close relatives.
What triggers dangerous heart rhythms in Brugada syndrome?
Fever is a common trigger, along with certain medications, excessive alcohol, and electrolyte imbalances. People with the condition are advised to treat fevers quickly and to check whether new medicines are safe for them.
How is Brugada syndrome treated?
High-risk people are usually treated with an implantable defibrillator that can detect and stop a dangerous rhythm with a shock. Avoiding known triggers and regular follow-up with a heart specialist are also important, and some people are managed with medication.
Can someone with Brugada syndrome live a normal life?
Many people do live full lives, especially when the condition is identified, triggers are avoided, and high-risk individuals are protected with a defibrillator. Ongoing care with a cardiologist helps keep the risk as low as possible.
References
- American Heart Association. Brugada syndrome.
- Mayo Clinic. Brugada syndrome — Symptoms and causes.
- National Institutes of Health, Genetic and Rare Diseases Information Center (GARD). Brugada syndrome.
- MedlinePlus, U.S. National Library of Medicine. Brugada syndrome.