An autosomal dominant defect of cardiac conduction that is characterized by an abnormal ST-segment in leads V1-V3 on the ELECTROCARDIOGRAM resembling a right bundle-branch block; high risk of VENTRICULAR tachycardia; or ventricular fibrillation; SYNCOPAL EPISODE; and possible sudden death. This syndrome is linked to mutations of gene encoding the cardiac sodium CHANNEL alpha subunit.


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