Brugada syndrome in a north Indian female-a case report.

ABSTRACT

The Brugada syndrome is an autosomal dominant disease with incomplete penetrance, which may cause syncope and sudden cardiac death in young individuals with a normal heart. It is characterized by an electrocardiographic pattern of complete or incomplete right bundle branch block and ST-segment elevation in leads V1-V3. Mutations in gene encoding for cardiac sodium channel SCN5A is linked to this syndrome in 20-25% patients. Several conditions producing Brugada-like ECG patterns should be borne in mind and excluded before making a diagnosis of the Brugada syndrome. The management of this syndrome is difficult as pharmacological agents are not universally effective. The mode of treatment recommended by the majority of cardiac electro-physiologists is implantation of a cardioverter defibrillator. We report the first case of a 40-year-old Indian lady who presented with a history of aborted sudden cardiac death with recurrent ventricular arrhythmias and an electrocardiogram (ECG) pattern of type 1 Brugada syndrome, and underwent successful implantable cardioverter defibrillator implantation.