Abstract

Ebstein's anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets.This condition often occurs in association with other congenital, structural, or conduction system disease including intracardiac shunts, valvular lesions, and accessory conduction pathways such as Wolff Parkinson White syndrome.It is also associated with a wide range of arrhythmias the commonest of which is Supra Ventricular Tachycardia (SVT).First degree atrioventricular (AV) block occurs in around 42% of patients with Ebstein's anomaly because of right atrial enlargement and structural abnormalities of the AV conduction system.We are reporting the rare occurrence of a case of Ebstein's anomaly who presented to us with complete atrioventricular block, he was successfully treated with single chamber permanent pacemaker implantation.

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