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Brugada syndrome
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Syncope, sudden cardiac death
Complications	N/A
Onset	Typically in adulthood
Duration	Lifelong
Types	N/A
Causes	Genetic mutations, primarily in the SCN5A gene
Risks	Family history, male gender
Diagnosis	Electrocardiogram, genetic testing
Differential diagnosis	N/A
Prevention	N/A
Treatment	Implantable cardioverter-defibrillator, quinidine
Medication	N/A
Prognosis	Variable, risk of sudden death
Frequency	Rare
Deaths	N/A

Brugada syndrome is a genetic disorder that results in abnormal electrical activity within the heart, leading to an increased risk of sudden cardiac death. It is characterized by a specific pattern on an electrocardiogram (ECG) and is often associated with ventricular fibrillation.

History

Brugada syndrome was first described in 1992 by the Brugada brothers, Pedro Brugada and Josep Brugada, who identified the distinct ECG pattern associated with the condition. Since its discovery, it has been recognized as a significant cause of sudden cardiac death in individuals with structurally normal hearts.

Genetics

Brugada syndrome is primarily inherited in an autosomal dominant manner, meaning a single copy of the mutated gene can cause the disorder. The most common genetic mutation associated with Brugada syndrome occurs in the SCN5A gene, which encodes the alpha subunit of the cardiac sodium channel. Mutations in this gene can lead to a loss of function of the sodium channel, affecting the heart's electrical activity.

Pathophysiology

The pathophysiology of Brugada syndrome involves abnormalities in the cardiac ion channels, particularly the sodium channels. This results in altered cardiac repolarization and conduction, predisposing individuals to ventricular arrhythmias. The characteristic ECG findings include a coved-type ST-segment elevation in the right precordial leads (V1-V3).

Clinical Presentation

Individuals with Brugada syndrome may present with a variety of symptoms, including:

• Syncope
• Palpitations
• Sudden cardiac arrest

Some individuals may remain asymptomatic, and the condition is often discovered incidentally during routine ECG screening.

Diagnosis

The diagnosis of Brugada syndrome is primarily based on the presence of characteristic ECG findings. These include:

• Coved-type ST-segment elevation in leads V1-V3
• Right bundle branch block pattern

In some cases, genetic testing may be performed to identify mutations in the SCN5A gene or other related genes.

Management

The management of Brugada syndrome focuses on preventing sudden cardiac death. Treatment options include:

• Implantable cardioverter-defibrillator (ICD): The most effective treatment for preventing sudden death in high-risk individuals.
• Quinidine: A medication that can help prevent arrhythmias in some patients.
• Lifestyle modifications: Avoidance of certain medications and reduction of fever, which can exacerbate the condition.

Prognosis

The prognosis of Brugada syndrome varies depending on the presence of symptoms and the risk of arrhythmias. Individuals with a history of syncope or cardiac arrest are at higher risk and may require more aggressive management.

Epidemiology

Brugada syndrome is considered a rare condition, with a higher prevalence in Southeast Asian populations. It is more common in males than females and typically presents in adulthood.

See also

• Long QT syndrome
• Sudden cardiac death
• Cardiac arrhythmia

External links

• Brugada Syndrome Foundation

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