What is Brugada Syndrome?
In Brugada Syndrome the underlying fault may be due to changes within the cells of the heart (ion channels). Ion channels alter the chemical balance of the cardiac cells by adjusting the amount of electrical charge to them, therefore, if the electrical properties of the cell are faulty this can result in a disturbance of the heart rhythm (arrhythmia). In Brugada Syndrome, the ion channel affect is the sodium channel. Brugada syndrome is referred to as a channelopathy, this is the term given to conditions where there are changes in genes that determind the ion channels. Your heart is otherwise structually normal.
In some cases, genes have been identified for Brugada syndrome, however, the list identified is not compelte. It is therefore not possible to absolutely rule-out the presence of Brugada Syndrome even if the genetic screening is negative. Please remember that if you are advised to have genetic screening, it may take some time to perform.
In Brugada Syndome, some patients may be at risk of developing fast heart rhythms which may result in blackouts or very rarely, sudden death. If the ventricles of the heart start beating at an abnormally fast rate, this results in the heart not working as efficiently. This can lead to weakness, dizziness, chest pain, shortness of breath or even collapse. However, it is important to remember that the majority of patients who have Brugada Syndrome do not experience arrhythmias and feel perfectly well.
It is very important that if you have a close family member diagnosed with the condition or dies young, that all remaining relatives are screen for Brugada Syndrome. If you doctor suspects that you may have Brugada Syndrome, they may advise you to have a simple test known as Ajmaline Challenge to confirm the diagnosis.
Why do I need an Ajmaline Challenge?
Your doctor has advised you to undergo an Ajmaline Challenge to exclude Brugada Syndrome. Ajmaline Challenges are a well-established clinical test. It is undertaken to see if you have a hidden form of Brugada Syndrome. The purpose of the test is to use a drug called Ajmaline to uncover the characteristic Electrocardiogram (ECG) changes of the syndrome. The characteristic of the ECG pattern of Brugada Syndrome is shown below.
What is an Ajmaline Challenge?
Ajmaline is primarily an anti-arrhythmic drug used to treat abnormal heart rhythms. However, due to the short-acting nature of this drug, there are better anti-arrhythmics available on the market. Therefore, Ajmaline is perfect for the purpose of trying to induce a Brugada ECG and because of it's short-acting nature, it leaves the body system quickly. Previously, Flecainide was used for this challenge, however, it is a long-acting drug and therefore ajmaline is better suited to this test. In patients with normal cardiac cells, Ajmaline has little or no effect on ECG characteristics.
Your doctor will administer the drug through a vein in your arm and record your ECG every three minutes. An ECG records the heart's electrical signals and therefore shows how your heart reacts to Ajmaline. By comparing ECGs before and after Ajmaline is administered, the doctor can collect detailed information about the potential cause of suspected arrthymia. The Ajmaline Challenge is undertaken in a ward setting with cardiac monitoring.
Is the Ajmaline Challenge safe?
Yes, the Ajmaline Challenge is safe. However, as with any procedure, there are potential risks. The risks will be fully explained by our doctors before you have your procedure. The Ajmaline Challenge is performed safely in both children and adults. In our experience complications associated with this procedure are very rare.
It is common (and harmless) to experience a metallic taste in your mouth while you are being given the Ajmaline. You may also experience some visual disturbances such as double or blurred vision. Such side effects usually resolve quickly once the infusion is completed. Very rarely the Ajmaline may cause your heart to go into a fast heart rhythm. When this does happen it often needs no treatment while we wait for the drug to wear off. Very rarely this heart rhythm may require urgent treatment with an electrical shock, chest compressions and/or an antidote to reverse the effects of the Ajmaline. For this reason, the test is performed in an environment where all these resources and experienced staff are available. No cases of death have ever been reported as a result of an Ajmaline challenge worldwide.
What can I expect during the test?
We ask that you do not eat or drink for four hours prior to your appointment time. Please inform us if you are diabetic. If you are taking any medications, you generally do not need to stop taking it, but please let us know which medications you are taking so we can discuss these with you.
On Arrival and Before the Ajmaline Challenge
On your arrival to the ward, you will be introduced to the nurse who will be looking after you. The nurse will talk to you and your family about your hospital admission and answer any questions you may have. Before the test begins, you will have a baseline electrocardiogram (ECG) recorded. A doctor will also see you and explain the procedure to you, they will then ask you to sign a consent form. This is to ensure you understand Ajmaline Challenge and its associated risks. If you have any worries or questions, please do not be afraid to ask. It is important to tell your nurse or doctor if you have any allergies or have had a previous reaction to drugs or other tests.
Before the procedure, a nurse will help you get ready. The doctor or nurse will need to insert a small needle (cannula) into a vein in your hand to give the doctor access to administer the Ajmaline. You will also be given a hospital gown to hear to allow us to record an ECG easily.
During the Ajmaline Challenge
It is unlikely you will experience any adverse sensations during the test. Although you may feel anxious during the procedure, the staff involved in your care are very aware of your potential anxieties. Your doctor and nurse will be with you to reassure you throughout the test. There will be equipment by your bedside to monitor your heart rhythm and record your blood pressure. You will remain awake during the test and be able to talk to us.
The nurse or doctor will connect you to the Ajmaline infusion which may sting a little and you may feel a little discomfort. You will then be connected to the ECG machine. Once the infusion is running, your doctor will record the ECG at one minute intervals for some time during and after the infusion. Sometimes patients comment on a metallic taste in the mouth, numbness around the lips or blurred vision. These side effects usually resolve quickly once the test is completed. If you do have any uncomfortable symptoms during the test such as chest pain, dizziness or shortness of breath, please tell your nurse or doctor immediately. Do not be alarmed that the doctor is looking closely at your ECGs during the test, it does not mean anything is wrong, they are simply noting if there are any changes.
What do patients commonly experience?
- Metallic taste in mouth
- Visual disturbances: blurred vision or double vision
Very rarely, Ajmaline can cause fast heart rhythms in some patients. If this happens, the doctor will need to correct your arrhythmia quickly using cardioversion. This procedure is used to treat heart rhythms that are irregular. Before you are cardioverted, you will be given a short-acting sedative to make you sleepy. Once you are asleep, a special machine called a defibrillator is used to send an electrical shock to the heart muscle to restore normal rhythm and rate.
After the Ajmaline Challenge is completed, you will be kept on a cardiac monitoring bed for a few hours after the procedure.
After the Ajmaline Challenge
There is a very low risk that you may experience arrhythmia or develop ECG changes of Brugada Syndrome after the Ajmaline has been administered, and hence, we will still monitor your heart rhythm for several hours after the drug has been given - even if the test is negative. We will wait for your ECG to return to normal before you are discharged.
As you will be monitored for several hours after the conclusion of the Ajmaline Challenge, the infusion and line cannula will be removed prior to discharge. The nurse will inform you of when you are able to eat and drink, while continuing to check your blood pressure and heart rate. If you feel any dizziness or palpitations after the test, please let the nurse know. The doctor will show your ECGs to your Consultant Cardiologist and discuss the findings of the test with you and your family.
When will I know the test result?
The doctor will discuss the ECG findings with the Consultant Cardiologist and provide you will a result and plan for ongoing treatment if required, prior to your discharge.
What treatment options are available?
There is currently no cure for Brugada Syndrome, but there are things you can do to reduce your risk of experiencing serious problems. Your Consultant Cardiologist will discuss the risks and benefits with you on an individual basis and your treatment options will be individualised. If you doctor confirms that you have Brugada Syndrome, the most important treatment is for you to avoid certain triggers that are associated with developing dangerous heart disturbances in Brugada Syndrome (see below).
If your risk of developing life-threatening arrhythmias is determined to be high, an implantable defibrillator (ICD) may be recommended. An ICD is also indicated when a patient has already suffered a dangerous arrhythmia. Some drugs are being used or investigated as treatment for rhythm abnormalities. Once a person is diagnosed with Brugada syndrome, the recommendation is for all first degree relatives to be screened.
It is important to know that many people who are diagnosed with Brugada Syndrome go on to live a long and happy life.
You can reduce your risk of developing a fast heartbeat by avoiding things that can trigger it. These include:
- a high temperature (fever) - if you develop a fever of 38
°C (100.4°F) or above, take painkillers such as paracetamol to bring it down; seek medical advice as soon as possible if this doesn't help. Click here for more information on how to manage fevers.
- drinking too much alcohol - avoid drinking lots of alcohol in a short space of time
- dehydration - seek medical advice if you have diarrhoea or vomiting that doesn't go away, as you may lose a lot of fluid and might require special rehydration drinks
- certain medicines - make sure any healthcare professional you see know you have Brugada syndrome and avoid medicines that trigger the condition unless they're recommended by a doctor. For over the counter medication, you can check with your pharmacist.
- hot baths and showers - you will still be able to have warm baths and showers, but nothing too hot such as saunas and hot Jacuzzis
- large carbohydrate meals - particularly in the evening.
When can I resume my normal activities?
You can resume your normal daily activities (walking, bathing, showering etc.) and return to work upon discharge from hospital.
Will I come back here for follow-up?
Upon discharge from the hospital, you will receive specific instructions by our nurses. Our doctors will write a detailed letter to your GP describing you hospital stay and treatment.