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Year : 2015  |  Volume : 2  |  Issue : 1  |  Page : 144-149

Brugada syndrome: A major cause of sudden cardiac death

1 Associate Professor, Department of Anatomy, BGC Trust Medical College, Chittagong, Bangladesh
2 Professor & Head, Department of Biochemistry, Chittagong Medical College, Chittagong, Bangladesh
3 Professor Molecular Biology & Genetics, Department of Anatomy, Bangabandhu Sheikh Mujib Medical University, Dhaka; Professor of Genetics and Molecular Biology of Bangabadhu Sheikh Mujib Medical University and Sub Project Manager, HEQEP CP 2057 of World Bank, Bangladesh

Correspondence Address:
Sanjoy Kumar Chakraborty
Associate Professor, Department of Anatomy, BGC Trust Medical College, Chandanysh, Chittagong
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Source of Support: None, Conflict of Interest: None

DOI: 10.5530/ami.2015.1.25

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In 1992 a new syndrome was described consisting of syncopal episodes or sudden death in patients with a structurally normal heart and an electrocardiogram characteristic of right bundle branch block with ST segment elevation in leads V1 to V3. Brugada syndrome is an autosomal dominant disorder. It has been shown to be associated with mutations in the gene (SCN5A) that encodes for the sodiumion channel in cardiac myocyte. Over 160 mutations of gene SCN5A have been identified. The incidence of the disease is difficult to estimate, but it causes sudden deaths of 5 per 10,000 inhabitants per year and involved much more frequently in people of Asian ancestry. Diagnosis can be easily made by means of genetic analysis and ECG. Recent data suggest that loss of the action potential dome in the right ventricular epicardium underlies ST segment elevation seen in this syndrome. Right ventricular epicardium is preferentially affected because of the predominance of transient outward current in this tissue. Antiarrhythmic drugs like amiodarone and beta-blockers do not prevent death in symptomatic or asymptomatic individuals. Though Implantation of an automatic cardioverter–defibrillator is the only recently proven effective therapy; Quinidine has been found to decrease Ventricular fibrillation and could prove to be a secured option of implantable cardioverter–defibrillator. However, researcher set focus on gene therapy that may offer an enduring cure in future years. The purpose of this brief review is to record the past highlights that have brought us to our present understanding of Brugada syndrome.

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