Abstract
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.
Highlights
The occurrence of sudden cardiac death (SCD), primarily due to malignant ventricular arrhythmias (VAs), affects approximately 350,000 people in the United States each year.[1]
This review describes available evidence-based risk stratification for SCD and current guideline-driven management strategies for the use of implantable cardioverter-defibrillator (ICD) in patients with congenital heart diseases (CHDs) and inherited arrhythmia syndromes
Relevant risk factors based on consensus recommendations include non-sustained ventricular tachycardia (VT), left ventricular dysfunction, QRS duration ≥ 180 ms, extensive right ventricular scarring on cardiac magnetic resonance imaging (MRI) and VT inducible at electrophysiology study (EPS).[7]
Summary
The occurrence of sudden cardiac death (SCD), primarily due to malignant ventricular arrhythmias (VAs), affects approximately 350,000 people in the United States each year.[1]. ICDs are able to successfully treat life-threatening VAs in patients with adult CHD and inherited arrhythmia syndromes.[8,9,10] 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] appropriate selection criteria for ICD implantation in these patients are poorly defined due to a paucity of randomized controlled trials in such patients. This review describes available evidence-based risk stratification for SCD and current guideline-driven management strategies for the use of ICDs in patients with CHD and inherited arrhythmia syndromes
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