Brugada syndrome

Also known as Brugada type idiopathic ventricular fibrillation, Pokkuri death syndrome, sudden unexplained nocturnal death syndrome, SUNDS, Bangungut, Dream disease

heart conduction disease characterized by abnormal ECG findings

Key facts

Other names: Sudden unexplained nocturnal death syndrome, bangungut, pokkuri death syndrome
Specialty: Cardiology
Symptoms: Passing out , sudden cardiac death
Usual onset: Adulthood
Causes: Genetics, certain medication
Risk factors: Family history, Asian descent, male
Diagnostic method: Electrocardiogram (ECG), genetic testing
Differential diagnosis: Romano-Ward syndrome , arrhythmogenic cardiomyopathy , Duchenne muscular dystrophy
Treatment: Watchful waiting , implantable cardioverter defibrillator (ICD)
Frequency: 1 per 2000
Deaths: 8% of sudden cardiac death

via Wikipedia infobox

Article

Brugada syndrome (BrS) is a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy. It increases the risk of abnormal heart rhythms and sudden cardiac death. Those affected may have episodes of syncope. The abnormal heart rhythms seen in those with Brugada syndrome often occur at rest, and may be triggered by a fever.

About a quarter of those with Brugada syndrome have a family member who also has the condition. Some cases may be due to a new genetic mutation or certain medications. The most commonly involved gene is SCN5A which encodes the cardiac sodium channel. Diagnosis is typically by electrocardiogram (ECG), however, the abnormalities may not be consistently present. Medications such as ajmaline may be used to reveal the ECG changes. Similar ECG patterns may be seen in certain electrolyte disturbances or when the blood supply to the heart has been reduced.