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Brugada syndrome

The Brugada syndrome is a genetic disease that is characterised by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death. It is also known as Sudden Unexpected Death Syndrome[1] (SUDS), and is the most common cause of sudden death in young men without known underlying cardiac disease in Thailand and Laos[2].

Although the ECG findings of Brugada syndrome were first reported[3] among survivors of cardiac arrest in 1989, it was only in 1992 that the Brugada brothers[4] recognised it as a distinct clinical entity, causing sudden death by causing ventricular fibrillation (a lethal arrhythmia) in the heart.

Genetics and pathophysiology

Type OMIM Mutation Notes
|- 
| B1 
alpha subunit of the sodium channel (SCN5A) Current through this channel is commonly referred to as INa. Gain of this channel leads to an unopposed Ito current (KCND2)
|- 
| B2 
GPD1L, Glycerol-3-phosphate dehydrogenase like peptide
|- 
| B3 
CACNA1C
|- 
| B4 
CACNB2 Beta-2 subnit of the voltage dependent L-type calcium channel
|- 
| B5 
KCNE3 which coassembles with KCND2 Beta subunit to KCND2. Modulates the Ito potassium outward current[5]
|- 
| B6 
SCN1B Beta-2 subunit of the sodium channel SCN5A[6]





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