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ID: 164
Category: Heart condition
CreatedBy: 1
UpdatedBy: 1
createdon: 13 Feb 2023
updatedon: 13 May 2023

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Author: Khoa Tran
Published Feb 13, 2023
Updated May 13, 2023

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Brugada Syndrome

History of Brugada Syndrome

Brugada syndrome is a rare but potentially fatal heart condition that was first discovered by two cardiologists, brothers Pedro and Josep Brugada, in 1992. The Brugada brothers were working at a hospital in Belgium at the time and reported on eight patients who had experienced sudden cardiac death without any apparent cause.

The Brugada brothers identified a distinct pattern on the electrocardiogram (ECG) of each of these patients, which they named the 'Brugada pattern'. This pattern is characterized by a specific type of abnormal heartbeat that can cause the heart to suddenly stop beating, leading to sudden cardiac death.

The discovery of Brugada syndrome was made possible by advancements in technology, specifically the invention of the standard 12-lead ECG. This technology allows doctors to measure the electrical activity of the heart from different angles, providing a detailed picture of its function.

The Brugada brothers were able to use the ECG to diagnose the condition in patients and identify the distinct pattern associated with the syndrome. This discovery has since been confirmed by numerous studies and has become an important tool for identifying patients at risk of sudden cardiac death.

In addition to the initial discovery of Brugada syndrome, other technologies have also played a role in further understanding and managing the condition, including genetic testing and implantable cardioverter-defibrillators (ICDs) that can detect and correct abnormal heart rhythms.

Modern Understanding of Brugada Syndrome

Brugada syndrome is a genetic heart condition that affects the electrical activity of the heart and increases the risk of sudden cardiac arrest. It is a relatively uncommon condition, affecting approximately 1 in every 2,000 to 5,000 people, and is most commonly diagnosed in people of Southeast Asian descent.

The symptoms of Brugada syndrome can be unpredictable and may not occur until late in life. In some cases, people with Brugada syndrome may not experience any symptoms at all, while others may experience symptoms such as:
- Fainting or near-fainting episodes: This can occur when the heart goes into an abnormal rhythm, causing a temporary lack of blood flow to the brain.
- Chest pain or discomfort: This can occur as a result of reduced blood flow to the heart, causing angina.
- Shortness of breath: This can occur as a result of heart failure, a condition in which the heart is unable to pump enough blood to meet the body's needs.

Diagnosis of Brugada syndrome typically involves an electrocardiogram (ECG), which can show the characteristic pattern associated with the condition. Other tests, such as an echocardiogram, a stress test, or an electrophysiology study, may also be performed to confirm the diagnosis and evaluate the severity of the condition.

Causes of Brugada Syndrome

Researchers have found more than 18 genes linked to the syndrome. They believe that Brugada Syndrome is inherited in a complex way, which means that many different genetic variations work together to cause it.

Treatments for Brugada Syndrome

Implantable Cardioverter Defibrillator (ICD): An ICD is a small device that is implanted under the skin of the chest and is connected to the heart through wires. It continuously monitors the heart rhythm and delivers an electrical shock to restore a normal heart rhythm if a life-threatening arrhythmia is detected.
Medications: Medications may be prescribed to manage symptoms and reduce the risk of complications in people with Brugada syndrome. These may include sodium channel blockers, such as quinidine, which can help prevent arrhythmias.
Lifestyle Changes: Lifestyle changes, such as avoiding triggers, maintaining a healthy weight, engaging in regular exercise, and managing stress, can also help manage Brugada syndrome and reduce the risk of complications.
Cardiac Ablation: In some cases, a procedure called cardiac ablation may be recommended. During this procedure, a catheter is threaded through a blood vessel to the heart, where it delivers radiofrequency energy to destroy small areas of heart tissue that are causing the arrhythmia.
Genetic Counseling: Since Brugada syndrome is a genetic condition, genetic counseling may be recommended for individuals with the condition and their family members. Genetic testing can help identify family members who may be at risk for developing the condition and allow for early detection and treatment.

The treatment plan for Brugada syndrome will depend on the individual's specific needs and risks. It is important to work closely with a healthcare provider who specializes in the management of Brugada syndrome to develop an individualized treatment plan.

Lifestyle Changes

While there is no cure for Brugada syndrome, there are several lifestyle changes that may help manage the symptoms and reduce the risk of complications.

- Avoiding Triggers: Certain medications, foods, and activities can trigger heart rhythm abnormalities in people with Brugada syndrome. Avoiding these triggers can help reduce the risk of complications. Examples of triggers include certain medications like antidepressants and some antiarrhythmic drugs, as well as activities like fever, excessive alcohol consumption, and strenuous exercise.
- Medication Management: Medications may be prescribed to help manage the symptoms of Brugada syndrome and reduce the risk of complications. It is important to take medication as prescribed and to notify your doctor if you experience any side effects or changes in your symptoms.
- Implantable Cardioverter Defibrillator (ICD): An ICD is a small device implanted under the skin that continuously monitors the heart rhythm and delivers an electric shock if an abnormal rhythm is detected. This can help prevent sudden cardiac death in people with Brugada syndrome who are at high risk of complications.
- Regular Monitoring: People with Brugada syndrome should receive regular checkups and monitoring from their healthcare provider to monitor the condition and ensure that any changes in symptoms or risk factors are addressed promptly.
- Maintaining a healthy weight: Excess weight can put additional strain on the heart, increasing the workload of the heart and making it more difficult for the heart to pump blood efficiently. This can lead to an increased risk of heart rhythm abnormalities and other complications in people with Brugada syndrome.
- Regular physical activity: Exercise helps to improve circulation, which can help improve the delivery of oxygen and nutrients to the heart and other organs. This can help reduce the risk of complications and improve overall heart health. 
- Quit smoking: Smoking cessation is also crucial, as smoking can increase the risk of heart disease and complications in people with Brugada syndrome.
- Reducing Stress: Stress can trigger heart rhythm abnormalities in people with Brugada syndrome, so finding ways to manage stress, such as through regular exercise, can help reduce the risk of complications.

It is important to work closely with your healthcare provider to develop an individualized treatment plan that is tailored to your specific needs and risks.

Symptoms

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Irregular heartbeat
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Chest pain
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Shortness of breath
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Fatigue
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Fainting / loss of consciousness
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Swelling in the legs, ankles, feet, or face (edema)

Confirmation Tests

- Echocardiogram
- Electrocardiogram (ECG)
- Electrophysiological (EP) testing and mapping

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