Brugada syndrome

Posted by e-Medical PPT Saturday, August 21, 2010
Brugada syndrome is a genetic disease that is characterised by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death by causing ventricular fibrillation in the heart.Approximately 20% of the cases of Brugada syndrome have been shown to be associated with mutation in the gene that encodes for the sodium ion channel in the cell membranes of the the myocytes.The gene, named SCN5A, is located on the short arm of the third chromosome (3p21).This results in transmural and epicardial dispersion of repolarization. The transmural dispersion underlies ST-segment elevation and the development of a vulnerable window across the ventricular wall, whereas the epicardial dispersion of repolarization facilitates the development of phase 2 reentry, which generates a phase 2 reentrant extrasystole that captures the vulnerable window to precipitate ventricular tachycardia and/or fibrillation that often results in sudden cardiac death.
Brugada syndrome has 3 different ECG patterns. Type 1 has a coved type ST elevation with 2 mm J-point elevation a gradually descending ST segment and a negative T-wave. Type 2 has a saddle back pattern with a least 2 mm J-point elevation and at least 1 mm ST elevation with a positive or biphasic T-wave.Type 3 has a saddle back pattern with less than 2 mm J-point elevation and less than 1 mm ST elevation with a positive T-wave.

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