Brugada syndrome: Clinical evaluation, diagnosis and treatment modalities: Review
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Date
2005
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Publisher
Turkiye Klinikleri
Access Rights
info:eu-repo/semantics/closedAccess
Abstract
Patients without demonstrable structural heart disease and an ECG pattern of right bundle-branch block with ST-segment elevation in leads V1 through V3 are at risk for ventricular fibrillation. The Brugada syndrome is a symptom complex involving these traits and including recurrent syncopal episodes followed by polymorphic ventricular arrhythmias or sudden cardiac death. Generally, there is no association with organic heart disease. It is diagnosed mostly in males in the fourth decade. Mutations in the SCN5A gene encoding voltage-linked Na+ channels on cell membranes have been postulated as the responsible pathophysiology. These gene mutations result in trans-membranous voltage gradients in myocardium due to early inactivation of Na+ channels. Such mutations are also thought to be responsible for certain of the inherited arrhythmogenic diseases (e.g., long QT syndrome, Lenègre disease, and sudden infant death syndrome), of which the Brugada syndrome is one. ST segment elevation might be unmasked with drug challenge in uncertain cases. Subjects at highest risk for cardiac sudden death are those with a familial history of sudden death and syncopal episodes. EPS must be performed on such patients. Patients manifesting ventricular arrhythmias (VT, VF) on EPS must be promptly managed. Amiodarone and/or beta-blockers do not protect against sudden death. Quinine may be used as medical management, but the ICD is the present treatment of choice. In this article, we discuss the diagnosis, clinical evaluation and treatment modalities of the Brugada syndrome. Copyright © 2005 by Türkiye Klinikleri.
Description
Keywords
Brugada Syndrome; Diagnosis; Therapeutics, Amiodarone; Beta Adrenergic Receptor Blocking Agent; Quinine; Voltage Gated Sodium Channel; Brugada Syndrome; Cell Membrane; Defibrillator; Electrocardiogram; Gene Mutation; Heart Electrophysiology; Heart Muscle; Heart Ventricle Arrhythmia; Heart Ventricle Fibrillation; Human; Long Qt Syndrome; Pathophysiology; Review; Risk Factor; Sex Difference; St Segment Elevation; Sudden Death; Sudden Infant Death Syndrome; Syncope
Journal or Series
Turkiye Klinikleri Journal of Medical Sciences
WoS Q Value
Scopus Q Value
Q4
Volume
25
Issue
4
