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• W2890948369 startingPage "187" @default.
• W2890948369 abstract "Brugada syndrome was first reported as a distinct entity in 1992. It is diagnosed by signature EKG changes including at least 2-mm J-point elevation with coved-type ST elevation and T-wave inversion in at least one right precordial leads (type I Brugada marker). Initially thought of as a rare entity, Brugada syndrome is now widely recognized as a common cause of natural death among young men as a result of ventricular arrhythmia occurring at rest, particularly during sleep. The etiology of this disease is likely multifactorial, with genetic predisposition playing an important role in the pathogenesis. Mutation in the sodium channel gene SCN5A is seen in up to 20–25% of patients with at least 17 additional genes were reported to be associated with the disease. However, mutation in a single gene could be implicated in less than 30% of patients with Brugada syndrome, and a recent study showing association of this disease with common single nucleotide polymorphism of SCN5A, SCN10A, and HEY2 pointed toward polygenic or oligogenic pattern of inheritance rather than a single gene defect. Despite initial reports of normal structural heart in majority of patients, recent reports showed frequent minor structural abnormalities, especially in the right ventricular outflow tract (RVOT) in these patients. Brugada syndrome is now viewed as a spectrum of cardiomyopathy as well as channelopathy. The pathophysiologic underlying cardiac arrhythmia in Brugada syndrome is still unresolved with continued debate on depolarization versus repolarization defect. Treatment is largely dependent on the symptom with implantable cardioverter-defibrillator (ICD) implantation indicated in patients with severe symptoms. Avoiding medications and/or conditions that predispose the patient to ventricular arrhythmia is advised in all patients. Quinidine, by blocking transient outward current, has been used with some success. RF ablation of the epicardial substrate in the RVOT has been shown to prevent recurrence of ventricular arrhythmia in severe cases." @default.
• W2890948369 created "2018-09-27" @default.
• W2890948369 creator A5076495472 @default.
• W2890948369 creator A5079800282 @default.
• W2890948369 date "2018-01-01" @default.
• W2890948369 modified "2023-09-26" @default.
• W2890948369 title "Brugada Syndrome: Current Perspectives" @default.
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