William Goodyer

Background: Limited data exist regarding implantable cardioverter defibrillator (ICD) usage in infants and toddlers. This study evaluates ICD placement indications, procedural techniques, programming strategies, and outcomes of ICDs in infants and toddlers. Methods: This is a single-center retrospective review of all patients ≤3 years old who received an ICD from 2009 to 2021. Results: Fifteen patients received an ICD at an age of 1.2 years (interquartile range [IQR], 0.1–2.4; 12 [80%] women; weight, 8.2 kg [IQR, 4.2–12.6]) and were followed for a median of 4.28 years (IQR, 1.40–5.53) or 64.2 patient-years. ICDs were placed for secondary prevention in 12 patients (80%). Diagnoses included 8 long-QT syndromes (53%), 4 idiopathic ventricular tachycardias/ventricular fibrillations (VFs; 27%), 1 recurrent ventricular tachycardia with cardiomyopathy (7%), 1 VF with left ventricular noncompaction (7%), and 1 catecholaminergic polymorphic ventricular tachycardia (7%). All implants were epicardial, with a coil in the pericardial space. Intraoperative defibrillation safety testing was attempted in 11 patients (73%), with VF induced in 8 (53%). Successful restoration of sinus rhythm was achieved in all tested patients with a median of 9 (IQR, 7.3–11.3) J or 0.90 (IQR, 0.68–1.04) J/kg. Complications consisted of 1 postoperative chylothorax and 3 episodes of feeding intolerance. VF detection was programmed to 250 (IQR, 240–250) ms with first shock delivering 10 (IQR, 5–15) J or 1.1 (IQR, 0.8–1.4) J/kg. Three patients (20%) received appropriate shocks for ventricular tachycardia/VF. No patient received an inappropriate shock. There were 2 (13%) ventricular lead fractures (at 2.6 and 4.2 years post-implant), 1 (7%) pocket-site infection, and 2 (13%) generator exchanges. All patients were alive, and 1 patient (7%) received a heart transplant. Conclusions: ICDs can be safely and effectively placed for sudden death prevention in infants and toddlers with good midterm outcomes.