Brugada syndrome: sudden death at any age

Brugada syndrome is an inherited, genetic disease that causes sudden death in young people, usually between 35 and 45 years of age but can cause sudden death at any age.

It was a disease that was discovered in 92 in 8 patients as a scientific curiosity, but as the years went by it became clear that it was a very prevalent disease in some areas of Southeast Asia where it can affect, in some areas of the Philippines, even 1 in 200 males.

It is a disease associated with sudden cardiac death, especially in men, which has a worldwide prevalence of 1 in 10,000 in the western population and is characterized by alterations at the level of the ion channels, which are those that control the electrical activity of the heart, and when they fail the person can develop cardiac arrhythmia and sudden cardiac death.

Since 1992 research has been carried out and hundreds of affected patients have already been found, the genetic basis associated with the disease has been found, practically 20 genes have been found that are already associated with this disease and therefore we can now have in our hands diagnostic mechanisms such as genetic tests to find patients at risk of suffering from this disease.

In addition, we have other diagnostic tools such as tests with flecainide, which is a drug that we give intravenously to provoke the arrhythmia, the electrocardiographic pattern of Brugada syndrome and we can also use the electrophysiological study to determine the risk.

Diagnosis of Brugada Syndrome

The diagnosis of Brugada syndrome is based mainly on the 12-lead electrocardiogram in which we can see a very characteristic shark-fin pattern that should be recognized by virtually all physicians who handle electrocardiograms. This is an electrocardiographic pattern that should make one quickly suspect the disease, the risk of sudden death and therefore the patient should be referred to a specialist in arrhythmias, a specialist in Cardiology, specialized above all in cardiac rhythm.

Is there a cure for Brugada syndrome? How can it be avoided or prevented?

The treatment of this disease is based mainly on the implantation of an automatic defibrillator because there are few drugs that work to prevent sudden death in these patients.

When we identify a patient, with the electrocardiographic pattern of Brugada syndrome, if this patient has had symptoms, this can obviously be a recovered sudden death or a loss of consciousness which is called syncope, a defibrillator is implanted in this patient. The only thing the defibrillator does is that when the patient develops an arrhythmia it gives him an electric shock so that this arrhythmia stops and the heart starts beating normally again. There is some medication such as quinidine that can be given to patients who cannot tolerate a defibrillator or who do not want a defibrillator or who are too young to have one. Quiniline has been shown to be somewhat effective in the treatment of Brugada syndrome. In any case, it is not a first choice treatment because it has not been shown to be 100% effective.

Finally, in the last 2-3 years, new techniques have appeared which are ablation techniques that consist of burning a part of the heart by means of a catheter that is inserted through the leg and burns the area of the heart that we believe is associated with the development of Brugada syndrome. By burning this area, the electrocardiographic pattern disappears, at least in the previous analyses or trials that have been carried out in some hundreds of patients, the electrocardiographic pattern does not appear when we give them the intravenous drug to cause Brugada syndrome and we do not induce arrhythmias, we cannot cause arrhythmias, when we carry out an electrophysiological study.

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These 3 details, the disappearance of the electrocardiogram, the fact that the electrocardiogram does not appear with the intravenous drug and that we cannot cause arrhythmias during the electrophysiological study, are 3 very important parameters that indicate that the risk of sudden death due to Brugada syndrome has practically disappeared. In any case, this is a very recent study in recent years and we obviously need a long-term assessment of these patients.

What are the risk factors for sudden death?

Well, in Brugada syndrome we have seen that there are some very clear risk factors, the first is obviously that a patient who has suffered a sudden death has a risk of developing a new episode of sudden death, so the first risk factor is to have suffered a recovered sudden death.

The second is having suffered a syncope, a loss of consciousness, therefore it is also associated with a higher risk of sudden cardiac death in patients with Brugada syndrome.

The third risk factor is having a positive electrophysiological study and being male, also have a higher risk of Brugada syndrome.

The risk factors that we try to prevent are those that can cause Brugada syndrome in these patients already diagnosed, such as avoiding medications that can cause the electrocardiographic pattern, these are medications for epilepsy, specific medications for arrhythmias, medications that we normally give to specialists and therefore the patient has to inform the physician about having Brugada syndrome. Finally, fever can also be a cause of arrhythmias in patients with Brugada syndrome, so we ask you to treat fever quickly with antipyretics.

What to do in case of cardiac arrest?

In a cardiac arrest the heart stops pumping blood, the person loses consciousness, stops breathing and therefore we have to restore the heart to normal rhythm through the use of an electric shock that we can give by means of an external defibrillator. The external defibrillator is placed following the drawing, the patches on the chest and if it is automatic it will work alone, if it is semiautomatic it will be giving orders and will simply give the order to press the button at the right time.

While we do not have a defibrillator, we have to perform cardiac massage by compressing the chest with both hands, 5 or 6 cm and combining 30 compressions with 2 insufflations. If the person does not want to perform insufflations, at least perform cardiac compressions. This means saving time, cardiac massage allows us to save precious time, precious minutes, so that the defibrillator has time to arrive to give the electric shock and recover the rhythm and also so that the ambulance can arrive and take the patient to the hospital.