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Brugada Syndrome

What is Brugada syndrome?
 

In Brugada syndrome the heart is structurally normal, but patients may be at risk of developing a fast heart rhythm due to changes within the ion channels of the heart. Ion channels alter the chemical balance of cardiac cells, by adjusting the amount of electrical charge to them. Therefore if the electrical properties of a cell are faulty this can result in a disturbance of the heart rhythm (arrhythmia). Patients who develop a fast heart rhythm may suffer from a blackout, or very rarely sudden death. The ion channel involved in Brugada syndrome is a sodium channel.

 

Some genes for Brugada syndrome have been identified but the list is not complete. It is therefore impossible to be sure that a patient does not have Brugada syndrome even if a genetic screening, with a blood test or mouth swab, is negative.


As noted above, genetic testing is at a very early stage for the diagnosis of inherited heart rhythm disorders. However, you and your family may be offered genetic testing if Brugada syndrome is diagnosed in a relative. If the faulty gene is discovered in the suspected case, and then also found in the relative, there is a risk that the relative could suffer symptoms because of Brugada syndrome. If the genetic test is negative, it may not rule out a genetic heart rhythm disorder. Your genetics consultant/counsellor will be able to give more extensive advice.

Patients may be at risk of developing a fast heart rhythm. Patients who develop a fast heart rhythm may suffer from a blackout or, very rarely, sudden death.

If your doctor suspects that you may have Brugada syndrome he will advise you to have a simple test known as the flecainide (or Ajmaline) challenge to confirm your diagnosis.


Flecainide is a drug that blocks sodium channels. As it blocks the faulty sodium channels it unmasks ECG changes in patients with Brugada syndrome.


Your doctor will administer the drug through a vein in your arm and record your ECG. The ECG will record how your heart reacts to the flecainide that allows the doctor to collect detailed information about the cause of your potential arrhythmia.


The flecainide challenge is safe, but as with any procedure there are potential risks. Complications associated with the procedure are very rare, can be treated and are rarely life threatening.

If the test result is negative, your doctor will consider your individual risk, and advise you if further tests are needed. It is likely that you will be able to go home the same day. However it is recommended that you do not drive, and that you have someone with you for the rest of the day after the test.


If the test is positive, and you are at risk of a fast heart rhythm developing, your doctor may suggest an electrophysiology study and ultimately you may be advised to have an implantable cardioverter defibrillator (ICD) fitted. An ICD will not prevent the arrhythmia but can treat it when one happens. If the test result is positive it is likely that you will be advised to remain in hospital until after these further tests.


Following your discharge from hospital you will be able to resume your normal daily activities, including returning to work.