Brugada syndrome

Brugada syndrome
Synonyms	Sudden unexplained nocturnal death syndrome, bangungut, pokkuri death syndrome
Pronounce
Field	Cardiology
Symptoms	Passing out, sudden cardiac death
Complications
Onset	Adulthood
Duration
Types
Causes	Genetics, certain medication
Risks	Family history, Asian descent, male
Diagnosis	Electrocardiogram (ECG), genetic testing
Differential diagnosis	Romano-Ward syndrome, arrhythmogenic cardiomyopathy, Duchenne muscular dystrophy
Prevention
Treatment	Watchful waiting, implantable cardioverter defibrillator (ICD)
Medication
Prognosis
Frequency	5 per 10,000
Deaths	8% of sudden cardiac death

Brugada syndrome (BrS) is a genetic condition characterized by abnormal electrical activity in the heart, leading to an increased risk of irregular heartbeats and sudden cardiac death. Individuals with Brugada syndrome may experience episodes of syncope (fainting), often triggered by rest or fever.

Overview

Brugada syndrome primarily affects the heart's rhythm, causing disturbances in the heart's electrical signals. These abnormalities can lead to life-threatening arrhythmias, including ventricular fibrillation and ventricular tachycardia, which can cause cardiac arrest and sudden death if not treated immediately.

Signs and Symptoms

Individuals with Brugada syndrome may experience the following symptoms:

Syncope (fainting) episodes, often during sleep or rest Palpitations Seizures Difficulty breathing Abnormal heart rhythms, typically revealed in an electrocardiogram (ECG) In some cases, the first indication of Brugada syndrome may be sudden cardiac arrest or death.

Causes

Brugada syndrome is a genetic condition, typically inherited in an autosomal dominant manner. Most cases are associated with mutations in the SCN5A gene, which codes for a sodium ion channel in the heart cells crucial for maintaining normal heart rhythm.

Diagnosis

Diagnosis of Brugada syndrome typically involves an ECG, which can detect characteristic patterns indicative of the syndrome. The condition can be further confirmed with genetic testing. Provocative drug testing using sodium channel blockers can also aid in diagnosis.

Treatment

The primary treatment strategy for Brugada syndrome focuses on the prevention of sudden cardiac death. Implantable cardioverter defibrillators (ICDs) are often used in high-risk individuals. Medications such as quinidine can also be used to reduce the risk of arrhythmias. Additionally, lifestyle modifications and avoidance of triggers such as fever and certain medications are recommended.

Prognosis

The prognosis for individuals with Brugada syndrome varies widely, depending on the severity of the condition and the individual's response to treatment. With appropriate management, individuals with Brugada syndrome can lead a normal life.

Epidemiology

Brugada syndrome affects both males and females, though it appears more commonly in males. It has a global distribution, with a higher prevalence reported in Southeast Asia.

Brugada syndrome Resources
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