ICD Indications - Professor Richard Kirk 2023

Professor Richard Kirk
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ICD Indications
The following information is taken from the 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary and should be consulted for detailed advice. ICD recommendations for DCM (HErEF), RCM (EFpEF) including HCM and for CHD are outlined below.
Dilated Cardiomyopathy
DCM or HFrEF is also referred to as "non ischemic" cardiomyopathy in the consensus statement. The incidence of SCD in pediatric patients with DCM is 1–5%, which is significantly less than that in adult patients which makes definitive evidence based advice difficult. Although some studies of adult patients with non ischemic cardiomyopathy and LVEF ≤ 35% suggest a survival benefit from an ICD there is no similar evidence in children. However, primary prevention ICDs may be considered for patients with syncope or severe impairment of left ventricular function despite optimal medical therapy (beta-blockers and afterload reduction) and after careful consideration of device-related complication risks, candidacy and timing of cardiac transplantation, and whether a wearable external defibrillator is a reasonable alternative.
Hypertrophic and Restrictive Cardiomyopathy
Hypertrophic Cardiomyopathy
HCM conventional risk factors include survival from a sudden cardiac arrest, spontaneous sustained VT, unexplained syncope, non-sustained VT, family history of  early HCM-related SCD, and massive left ventricular hypertrophy. While a left ventricular wall thickness ≥30 mm is considered a risk factor in adults a z score ≥5 is associated with an increased risk of SCD in children.

Restrictive Cardiomyopathy
Many causes of RCM include sarcomere abnormalities resulting in overlap with the HCM phenotype and they are at risk of SCD. However, RCM patients may not display thickening of the intraventricular septum and so ICD implantation may be appropriate in those who present with heart failure or unexplained syncope regardless of septal thickness.
Congenital Heart Disease
ICDs are commonly placed for both primary and secondary prevention in patients with CHD. Appropriate ICD shock rates of 3–6% per year have been shown with an increased frequency of shocks for secondary prevention indications. Anti-tachycardia pacing has been shown to be effective in VT termination and reducing ICD shocks. Patients with CHD receiving an ICD have an increased rate of complications as high as 26–45%, as well as a high rate of inappropriate  shocks. Note also that ICD implantation can be especially challenging in patients with CHD due to anatomic complexity, intracardiac shunts, or limited vascular access.  
Further Reading
Last Updated: April 2023
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