Inherited Electrical Disorder

Brugada Syndrome

Brugada Syndrome is an inherited electrical heart condition associated with characteristic ECG patterns and, in some people, an increased risk of ventricular arrhythmias. Many people live with it for years without symptoms.

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Overview

What Is Brugada Syndrome?

Brugada Syndrome is a disorder of the heart’s electrical system. It belongs to the group of inherited rhythm conditions, sometimes called channelopathies, which affect how electrical signals are produced in heart cells.

In many people the structure of the heart appears normal on routine imaging. The concern relates to the electrical behaviour of the heart, which in some people may be linked to a higher risk of dangerous ventricular rhythms.

The condition varies widely. Some people never have symptoms, while others are diagnosed after fainting or after a family member is assessed. It is also described on the broader Inherited Arrhythmia Syndromes page.

Electrical signalling

Electrical Basis

Each heartbeat depends on electrical charge moving in and out of heart cells through tiny channels. These channels open and close in a carefully timed sequence.

In Brugada Syndrome, the activity of some of these channels is altered in parts of the heart. This can change the electrical pattern recorded on an ECG and, in some people, may make the heart more susceptible to abnormal rhythms.

The details of this process are complex and differ between people. Your care team can explain what is relevant to you.

Recording the pattern

ECG Patterns

A characteristic pattern may be seen in the right-sided chest leads of the ECG, often called the right precordial leads. The pattern can come and go from one recording to another, and it can be influenced by factors such as fever.

Because of this, a single ECG tracing is not used on its own to confirm the diagnosis. Specialists consider the pattern together with the clinical history, family history and other findings.

Presentation

Symptoms

People with Brugada Syndrome may experience:

  • no symptoms at all, with the pattern found on an ECG done for another reason
  • fainting or near-fainting
  • palpitations
  • symptoms that occur at night or during rest, in some people
  • ventricular arrhythmias, in selected patients

Symptoms can have many causes, and having them does not mean a person has Brugada Syndrome. They should still be assessed.

If you faint suddenly or have severe symptoms, seek urgent medical help.

Family

Family History

Because Brugada Syndrome can be inherited, a history of unexplained fainting, rhythm problems or sudden cardiac events in relatives may be relevant. It can prompt specialists to consider the condition or to offer family assessment.

Family history alone does not confirm the diagnosis, and a normal family history does not rule it out. The condition may also be found in a person with no known family history.

Things to be aware of

Fever and Other Triggers

Fever can influence the ECG pattern in Brugada Syndrome and, in some patients, may affect clinical risk. Other factors, including some medicines and substances, may also be relevant for some people.

Advice differs from person to person. Please talk with your specialist about what applies to you, including what to do if you develop a fever. Do not start or stop any medicine unless your treating team tells you to.

Assessment

Diagnosis

Diagnosis is a clinical decision made by a specialist. It may take several sources of information into account:

  • the ECG, including repeated or specialised recordings where appropriate
  • your clinical history and symptoms
  • your family history
  • other tests where appropriate

Specialised ECG assessment is arranged by your care team when needed. The approach is individual, so your specialist will explain what applies to you.

A general outline

Assessment Pathway

This is a general outline. Steps differ from person to person.

History

Symptoms and family history are discussed.

ECG

The pattern is recorded and reviewed.

Further tests

Added where appropriate.

Risk review

Findings are considered together.

Plan

Follow-up is agreed with you.

Genes

Genetic Assessment

Genetic testing may be considered in some people, particularly where a diagnosis is established or the family history is relevant.

  • Not every person with Brugada Syndrome has an identifiable genetic variant.
  • A result needs careful interpretation by a specialist, because some findings are uncertain.
  • Genetic results may also be relevant to relatives, so counselling is often helpful.

Understanding risk

Risk Assessment

Risk differs a great deal between people. Specialists consider several factors together, such as symptoms, the ECG pattern, family history and other test results.

Most people with Brugada Syndrome are managed with follow-up and advice. Your care team will discuss what your own assessment means for you. No single feature gives the full picture.

Testing

Electrophysiology Study

An electrophysiology study (EPS) uses catheters to record and test the heart’s electrical behaviour. In Brugada Syndrome it may have a role in selected circumstances, as part of a wider risk assessment.

It is not required for everyone with Brugada Syndrome. Whether it is helpful depends on your situation, and your specialist will explain if it is being considered.

Protection

Implantable Cardioverter-Defibrillator (ICD)

An implantable cardioverter-defibrillator (ICD) is a device that can monitor the heart rhythm and treat a dangerous ventricular rhythm if one occurs.

ICD therapy may be considered in selected patients who are judged to be at higher risk. It is not needed by everyone with Brugada Syndrome. The decision is made individually after discussion with your care team.

The ECG Pattern

Type 1 Brugada Pattern and Why It Matters

The diagnostic ECG feature is a coved ST-segment elevation in the right chest leads, known as the type 1 pattern. It can be constantly present, or appear only intermittently, for example with fever or after a provocation test.

Other patterns may suggest Brugada syndrome but are not diagnostic by themselves. Interpretation requires specialist experience, as the pattern can vary from day to day.

Risk

How Risk Is Assessed

Most people with a Brugada pattern never experience a dangerous rhythm. The goal is to identify those at higher risk.

Symptoms

Fainting, documented ventricular arrhythmia or previous cardiac arrest weigh heavily.

ECG pattern

Whether the type 1 pattern is spontaneous or only provoked.

Family history

Sudden unexplained death in young relatives.

Selected patients may have electrophysiology study, genetic testing or ECG monitoring.

Daily Life

Fever, Medicines and Precautions

Fever

Fever can unmask or worsen the pattern. Prompt treatment of fever, for example with an antipyretic, is commonly advised.

Medicines to avoid or use with caution

Some drugs, including certain anti-arrhythmics, psychiatric and anaesthetic medicines, may provoke the pattern. Ask your cardiologist for a current list, and tell any doctor you see that you have Brugada syndrome.

Other triggers

Large meals, alcohol excess and electrolyte disturbances may matter in some patients.

These are general themes only. Your own precautions should be set by your cardiologist.

Long-Term

Clinical Follow-Up

Follow-up is individual. It may include periodic ECGs, review of symptoms and family evaluation. Patients considered at higher risk may be offered an implantable defibrillator or, in selected situations, ablation targeted at the right ventricular outflow tract.

Ongoing care

Follow-Up

Many people have regular specialist follow-up. It may include a review of symptoms, repeat ECGs and discussion of any changes in health or family history.

The type and frequency of follow-up are decided individually. Let your care team know if you notice new symptoms, such as fainting.

Questions

Frequently Asked Questions

No. It is an electrical condition. The heart structure and the coronary arteries are often normal.
Not necessarily. Many people are managed with follow-up and advice. Your specialist will explain the approach for you.
Yes. The pattern can vary between recordings, which is one reason a single ECG is not used alone.
Fever can influence the ECG pattern and, in some patients, risk. Ask your specialist what applies to you.
This may be recommended in some families. Your care team can advise whether family assessment is appropriate.
Not everyone does. ICD therapy may be considered in selected higher-risk patients after discussion with your care team.
No. It may be considered in some people, and not everyone has an identifiable variant.
Many people do. Your specialist can give advice on your own circumstances, including activity and follow-up.
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