Shahzad Raza, Abdul Hakeem, Salman Moinuddin, Rahat Noor, Umar Rasool Malhi, Syed Razi.
An insight into the Brugada syndrome.
Pak J Cardiol Jan ;16(2):67-80.

In 1992, on the basis of a small patient series, the brothers Pedro and Josep Brugada described a new syndrome characterized by syncopal episodes and/or sudden death in young and otherwise healthy adults, and less frequently in infants and children with a structurally normal heart and a characteristic electrocardiogram (ECG) pattern of right bundle branch block (RBBB) with ST segment elevation in leads V1 to V3. The ECG manifestations of Brugada syndrome (BS) are often dynamic or concealed and may be unmasked or modulated by sodium channel blockers, a febrile state, vagotonic agents, alpha-adrenergic agonists, beta-adrenergic blockers, tricyclic or tetra cyclic antidepressants, a combination of glucose and insulin, hypo and hyperkalemia, hypercalcemia, and alcohol and cocaine toxicity. Patients with a spontaneously appearing Brugada ECG have a high risk for sudden arrhythmic death secondary to ventricular tachycardia/ fibrillation. Recent genetic studies have confirmed the genetic heterogeneity of the disorder. Mutations of the cardiac sodium channel SCN5A have been detectable in <20% of patients with Brugada syndrome. Antiarrhythmic drugs appear to be of little use in prolonging survival and in preventing recurrences of ventricular arrhythmias. To date, implantable cardioverter defibrillator remains the best therapy to prevent sudden death in these patients. In recent years, an exponential rise in the number of reported cases and a striking proliferation of articles defining the clinical, genetic, cellular, ionic, and molecular aspects of the disease have been published. This article summarizes the current understanding of pathophysiology and clinical features of BS, risk stratification and diagnostic criteria and current clinical management.

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