What Is Brugada Syndrome?
In 1992, two brothers, Joseph and Pedro Brugada, discovered this disease. It was causing sudden death by ventricular fibrillation.
Brugada syndrome is an uncommon but occasionally hereditary cardiac rhythm disorder (arrhythmia) that can be fatal.
Individuals who have Brugada syndrome are more likely to experience abnormal cardiac rhythms that start in the ventricles, the bottom chambers of the heart.
Preventive methods for Brugada syndrome treatment include lowering the temperature and avoiding drugs that could cause the arrhythmia. An implantable cardioverter-defibrillator (ICD) is a medical device that is necessary for some individuals with Brugada syndrome.
What Is the Brugada ECG Pattern?
When an electrocardiogram is taken, a specific characteristic pattern is seen in the ECG, which resembles Brugada, but actually, the person has no underlying disease. This is called the Brugada ECG pattern.
How Prevalent Is Brugada Syndrome?
This disease was more prevalent in people of Asian origin than the American and European populations, of which type 2 and 3 ECG patterns are most common. The average age of patients who die from this disease is around 35 to 40 years.
What Causes Brugada Syndrome?
Brugada syndrome is mainly caused by a genetic mutation of a gene known as SCN5A. It is the gene that encodes sodium ion channels in the cell membrane of heart muscles. Only about 20 % of patients with this disease have a mutation in this gene. And some other patients have a defect in other genes.
It can either be genetic or acquired; it can either be inherited from a parent, or a congenital disability can occur in that individual without any inheritance. Even with a defective gene, there can be times when Brugada syndrome does not cause any symptoms and remains inactive.
Any of the following can unmask Brugada syndrome:
-
Antidepressants.
-
Illegal drugs.
-
Antipsychotics.
-
Problems with electrolyte balance.
-
Conditions that cause fever.
What Are the Signs and Symptoms of Brugada Syndrome?
The following are the signs and symptoms of Brugada syndrome:
-
Cardiac arrest and syncope, which occur primarily in the time of rest or sleep.
-
ST-elevation leads V1 to V3, which is most noticeable during a routine electrocardiogram.
-
Fever is often the trigger.
-
Blackouts.
-
Chest pain, dizziness, palpitations, and breathlessness may be seen occasionally.
-
Rapid heart rate.
What Increases the Risk of Developing Brugada Syndrome?
The following factors increase the risk of getting Brugada syndrome:
-
Brugada syndrome has a gender predisposition, with men more commonly affected than women. The incidence rate is about eight to ten times higher in men as compared to women. This has been attributed to the presence of the male hormone, testosterone.
-
Also, Brugada syndrome is more prevalent in people of Asian descent than individuals of another origin.
-
Having a family history of Brugada syndrome.
-
Fever, although it does not cause Brugada syndrome, can act as a triggering factor and cause fainting and sudden cardiac arrest in individuals with Brugada syndrome. This is more common in children.
How Is Brugada Syndrome Diagnosed?
1. ECG (Electrocardiography):
ECG shows a coved-type ST elevation in leads V1 to V3.
2. Genetic Testing:
People with a family history of sudden death should be genetically tested for a mutation in SCN5A.
3. Laboratory Test:
Check for serum potassium and calcium levels in patients with ST-elevation, as an increase in potassium and calcium can generate ECG patterns similar to Brugada syndrome.
4. Echocardiogram:
An echocardiogram helps in diagnosing Brugada syndrome with the help of sound waves. It also helps in ruling out structural problems of the heart.
5. Electrophysiology Test:
When symptoms of Brugada syndrome or cardiac arrest occur, electrophysiology tests help detect abnormality in heart rhythm. A catheter is inserted into the vein through which electrodes are inserted. It helps in diagnosing irregular heartbeats.
How Is Brugada Syndrome Treated?
The risk of developing abnormal heart rhythm can be reduced by:
-
Antipyretic drugs are to be taken soon after a fever has been diagnosed.
-
Competitive sports should be avoided.
-
Drugs taken for heart problems or antidepressants increase the risk of developing heart rhythm problems and should be avoided.
Surgical and Other Treatment Methods:
-
Implantable Cardioverter-Defibrillator - The most effective treatment at this moment is by implanting an automatic implantable cardioverter-defibrillator. It is a small device operated with the help of a battery and helps in monitoring heart rhythm. When an abnormality is detected, electrical shocks help control abnormal heart rate.
-
Catheter Ablation - When an implantable cardioverter-defibrillator is not adequate, radiofrequency catheter ablation is used. A catheter is inserted, which destroys the heart tissues that cause heart rhythm problems by delivering high energy.
-
Drug Therapy - Along with implantable cardioverter-defibrillators, Quinidine may be used to detect abnormal heart rhythms.
What Are the Risk Factors of Brugada Syndrome?
The following are risk factors for Brugada syndrome:
-
History of Brugada syndrome in the family. This illness is frequently inherited and passed down through families. A person is more likely to develop Brugada syndrome if they have family members who do.
-
Being a man. Brugada syndrome is diagnosed in men more often than in women.
-
Race. Asians experience Brugada syndrome more commonly than those of other races.
-
High temperature. While fever is not the cause of Brugada syndrome, it can aggravate the heart and induce fainting or sudden cardiac arrest in those who have it, particularly in young patients.
What Are the Complications Associated With Brugada Syndrome?
When Brugada syndrome exhibits complications, it is recommended to seek immediate medical attention. The following are the complications of Brugada syndrome:
-
Fainting - With Brugada syndrome, fainting is not a common medical condition and requires immediate medical care.
-
Sudden Cardiac Arrest - It is characterized by the sudden loss of heart function, problems in breathing, and loss of consciousness. A fatality occurs when adequate medical help is not sought at the right time. However, it most commonly occurs in sleep, so identifying and preventing it is difficult.
Can One Prevent Brugada Syndrome?
Brugada syndrome cannot be prevented as a whole; however, specific precautionary measures to reduce the risk of complications can be taken. Early detection helps in taking adequate preventive steps. When there is a family history of the disease, genetic testing can help diagnose or determine the risk of Brugada syndrome.
Conclusion
Arrhythmia, an uncommon but potentially fatal cardiac rhythm problem, can occasionally be inherited and is known as Brugada syndrome. The ventricles, the heart's lower chambers, are more likely to start abnormal cardiac rhythms in people with Brugada syndrome. Preventive steps including lowering temperature and avoiding drugs that could cause the arrhythmia are part of the treatment for Brugada syndrome. An implantable cardioverter-defibrillator, or ICD, is a necessary medical device for some individuals with Brugada syndrome.
