Brugada Syndrome (BrS) is an electrical disorder of the heart which results from faulty electrical channels in the cardiac cells. 

Patients with BrS have an increased risk of developing a dangerous heart rhythm disorder, Ventricular fibrillation or VF. BrS is rare and affects about 1 in 2000 of UK population. There is an increased prevalence in those from South East Asian communities where it affects about 1 in 400.

BrS appears to be more common in men (8:1) who are also more likely to have symptoms related to Brugada Syndrome. 

BrS is an inheritable (genetic) condition and you have a 50% chance of having BrS if one of your parents has the condition 

Most patients with BrS have no symptoms.

However some people experience:

1) Blackouts or a collapse

2) Fits (seizures)

3) Palpitations - Awareness of rapid heartbeat

Symptoms can be brought about by triggers – certain prescription medication, high temperature, drinking lots of alcohol, or dehydration.

In some cases the sudden death of a relative can be the first time Brugada syndrome is identified 

Those with the distinctive ECG pattern are diagnosed with Brugada Syndrome. This ECG pattern can be “spontaneous” – present without provocation, or it can be ”concealed” – requires a drug infusion to bring out the ECG pattern – Ajmaline or Flecainide.

Some patients can have an intermittently spontaneous pattern which can sometimes be brought out during a fever

In most patients with Brugada syndrome the heart appears to be “normal” even on detailed cardiac imaging

Although Brugada syndrome is a genetic disorder a genetic blood test can only identify the faulty gene in about 20-25% of patients with the condition. 

There is no cure for Brugada Syndrome, the main focus of treatment is to reduce the risk of a cardiac rhythm problem.

All patients require lifestyle advice

1) Avoidance of certain drugs – brugadadrugs.org

2) Prompt treatment of fever

3) Maintain good hydration

4) Routine follow up with a cardiologist

5) Avoid heavy meals at before going to sleep

High risk patients may require cardiac defibrillators – if the likelihood of them having a cardiac arrhythmia is felt to be high.

"How long have I had this & why has it not been picked up before?"

Brugada syndrome is inherited disorder so we have the condition at birth HOWEVER it does not develop until early adulthood

Children <16 are unlikely to show signs of Brugada Syndrome on their ECG’s

The ECG for Brugada syndrome is acquired in a different location across the chest to a normal ECG and therefore can often be hidden

Some patients require a drug challenge to bring out the pattern

The condition is rare – there is greater awareness now but in the past this may have been missed

"How will it affect my children?"

Children of an effected person have a 50% chance of inheriting the condition.

This does not mean that they will ever show signs of the condition

Children <16 are not normally affected by Brugada Syndrome, but we advise the lifestyle advice mentioned previously

Children & young adults under the age of 18 may have falsely negative Ajmaline tests and we advised waiting until they are older for this to be done in most cases

"How come my brother has a defibrillator and I don’t?" 

Different members of the family may have different degrees of risk based on the severity of the condition in them

Once we make a diagnosis of Brugada Syndrome we will try to estimate your risk based on your ECG and presence of symptoms

If we feel you are high risk it is likely that we will suggest a defibrillator

"Why can’t all patients just have defibrillators?" 

Defibrillators are associated with risk and risks from the procedure may outweigh the benefits. Risks include, 

1) Infections

2) Incorrect firing

3) Damage to vein, lung, heart during implantation

A defibrillator may also impact on other aspects of your life - psychological consequences, potential driving restrictions, issues obtaining life or holiday insurance for example.

"Can I play sport with Brugada Syndrome?"

Issues during sport are not common with Brugada syndrome

However – dehydration and excessive heat can trigger abnormal hearth rhythms in some cases

Ensure you are well hydrated and wear appropriate clothing to prevent high body temperatures

"Is it dangerous to have sex if you have Brugada Syndrome?" 

Not at all!

What is the risk to me during pregnancy? 

Patients with Brugada syndrome are generally safe during pregnancy

It is important you inform your midwife that you have Brugada syndrome incase you require any medication during delivery

Your pregnancy team will probably make contact with your cardiologist during your pregnancy for advice – don’t be afraid to ask/insist on this.

"My genetic test was negative – does this mean I don’t have Brugada Syndrome?"

The genetics of Brugada syndrome is complex and there is a lot we don’t know

We only identify the faulty gene in 20-25% of people with the condition.

In the other 75-80% we simply can’t find the causative gene

More research is needed to expand our knowledge of the genes that are involved in Brugada syndrome

"Where can I find more information about Brugada syndrome?" 

Cardiac Risk in the Young

British Heart Foundation

SADS UK