Title: Case of Brugada Syndrome
Authors: Dr Rajat Shankarrao Dalal, Dr Anil Modak, Dr Tanuja Manohar, Dr Nalini Humaney
DOI: http://dx.doi.org/10.18535/jmscr/v4i5.05
Abstract
In 1992 Brugada et al. described clinical entity associated with ST segment elevation in precordial leads (V1 to V3) with incomplete or complete Right Bundle Branch Block and susceptibility to ventricular tachyarrhythmia and sudden cardiac death has been described. The disease is now know as “Brugada syndrome” (BrS). The prevalence of Brugada syndrome is unknown as mostly is goes undiagnosed. Common presentation of Brugada syndrome is syncope typically occuring at rest or during sleep (mostly seen in third or fourth decade of life). Brugada syndrome can be life threatening in some cases as tachycardia does not terminate and it may degenerate into ventricular fibrillation and leads to sudden cardiac death. Brugada syndrome is an autosomal dominant pattern of inheritance. In approximately 20% of the cases BrS is caused by mutations in the SCN5Agene on chromosome 3p21-23, encoding the cardiac sodium channel. SCN5A is a protein involved in the control of myocardial excitability.Sodium channels are heat sensitive so fever can also unmask Brugada syndrome. The use of the implantable cardioverter defibrillator (ICD) is the only therapeutic option of proven efficacy for primary and secondary prophylaxis of cardiac arrest.
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