Abstract

t z p c b t t t a Introduction Advances in cardiac care of the young have given rise to a growing and aging population of patients with congenital heart disease. Despite remarkable improvements in overall survival, sudden cardiac death remains the most common cause of late mortality. As a result, implantable cardioverter-defibrillators (ICDs) are increasingly used in this heterogeneous patient population. Tetralogy of Fallot and transposition of the great arteries are the most prevalent subtypes of congenital heart disease in ICD recipients. Common to this young population is the high rate of lead-related complications and inappropriate shocks. In a multicenter study of patients with tetralogy of Fallot, 25% received inappropriate shocks, predominantly due to sinus or supraventricular tachycardia. Although a similar proportion of patients with transposition received inappropriate shocks (24%), 62% were due to oversensing or lead dysfunction. Herein, we offer 10 practical tips for optimizing ICD programming in patients with congenital heart disease (Table 1) in view of reducing inappropriate and potentially avoidable shocks.

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