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

Some congenital heart diseases (CHDs) and inherited arrhythmia syndromes are associated with an increased risk of sudden cardiac death (SCD). Appropriate selection criteria for implantable cardioverter-defibrillator (ICD) implantation in these patients are poorly defined due to a paucity of data available from randomized clinical trials, leading to current guidelines relying more on non-randomized studies and expert opinions to make their recommendations. This review describes available evidence-based risk stratification methods for identifying patients at risk for SCD, as well as current guideline-driven management strategies for the use of ICDs in patients with CHD and inherited arrhythmia syndromes.