The Brugada syndrome is a genetic disease that is manifest by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death. It is also known as Sudden Unexpected Death Syndrome1 (SUDS), and is the most common cause of death in the young in Thailand and Laos2. more...
First described in 19923, the Brugada syndrome causes sudden death by causing ventricular fibrillation (a lethal arrhythmia) in the heart.
Genetics and pathophysiology
Brugada syndrome is due to a mutation in the gene that encodes for the sodium ion channel in the cell membranes of the muscle cells of the heart (the myocytes). The gene, named SCN5A, is located on the short arm of the third chromosome (3p21). This condition is inherited in an autosomal dominant pattern.
In some cases, the disease can be detected by observing characteristic patterns on an electrocardiogram, which may be present all the time, or might be elicited by the administration of particular drugs. The pattern seen on the ECG is persistent ST elevations in the electrocardiographic leadsV1-V3 with a right bundle branch block (RBBB) appearance with or without the terminal S waves in the lateral leads that are associated with a typical RBBB. A prolongation of the PR interval (a conduction disturbance in the heart) is also frequently seen.
The cause of death in Brugada syndrome is ventricular fibrillation. While there is no treatment modality that prevents ventricular fibrillation from occurring in this syndrome, treatment lies in termination of this lethal arrhythmia before it causes death. This is done via implantation of an implantable cardioverter-defibrillator (ICD), which continuously monitors the heart rhythm and will defibrillate an individual if ventricular fibrillation is noted.
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