The Use of Implantable Cardioverter-defibrillators in the Prevention of Sudden Cardiac Death: A Focus on Congenital Heart Disease and Inherited Arrhythmia Syndromes

Abstract

heart disease (CHD) is increasing because medical and surgical care received during childhood has improved, resulting in an additional cohort at risk for malignant arrhythmias. SCD accounts for 20% to 25% of mortality in the adult CHD population. 5-7 ICDs are able to successfully treat life-threatening VAs in patients with adult CHD and inherited arrhyth-mia syndromes. 8-10 However, ICD use in this generally younger patient population is complicated, with high rates of device-related complications occurring over many decades of use, including inappropriate shocks, device-related infections, and lead displacement or failure. 11 Furthermore, appropriate selection criteria for ICD implantation in these patients are poorly defined due to a paucity of randomized controlled trials in such patients. Current professional guidelines therefore rely on data from non-randomized studies and on expert opinion. 11 This review describes available evidence-based risk strat-ification for SCD and current guideline-driven management strategies for the use of ICDs in patients with CHD and inherited arrhythmia syndromes.