Abstract Background Implantable cardioverter defibrillators (ICDs) are an important therapy for prevention of sudden cardiac death (SCD). Data on transvenous (TV) ICD therapy rates, complications, and system survival in children ≤ 21 years is limited by absolute numbers and inclusion of adults with congenital heart disease. Purpose To determine ICD therapy rates and complications in pediatric patients ≤ 21 years of age. Methods A systematic review and meta-analysis were conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Medline, Embase, PubMed Ahead-of-Print and in-process records, and Cochrane Database of Systematic Reviews were searched for full-text articles on pediatric ICD. Articles were assessed for inclusion by two authors via a consensus process; the exclusion criteria included: case reports, review articles, abstracts, editorials, articles not in English, articles solely on epicardial or subcutaneous leads, articles on unrelated topics, articles with <50% ICD leads, and articles with any patients >21 years old. Data were extracted by 1 author and verified by a 2nd author. Meta-analysis of aggregate data was performed, where possible. Random effects models were used for pooled inferences. All reported rates are annualized rates based on reported follow-up times in each study. Results Of 1325 articles, 23 met all inclusion and no exclusion criteria (Figure) representing 1700 patients implanted from 1987-2020. The average age of patients was 12.8 years (95% confidence interval [CI]: 11.9 – 13.7) with weight of 51.1 kg (95% CI: 42.2 – 60.0) (Table). Primary prevention was the indication in 56.4 % (95% CI: 45.0% – 67.7%). Only 14.8% (95% CI: 10.6% – 19.0%) of the patients had congenital heart disease while 22.6% (95% CI: 15.8% – 29.3%) had long QT syndrome. The annualized rate of appropriate shock was 7.0% (95% CI: 5.6% – 8.8%) and the rate of inappropriate shock was 4.8% (95% CI: 3.8% – 6.0%). Annualized rates of reported adverse events included lead failure: 2.3% (95% CI: 1.6% – 3.3%) and infection: 0.8% (95% CI: 0.4% – 1.6%), which could have led to system revision: 4.4% (95% CI: 2.1% – 9.4%). The all-cause mortality rate was 0.8% (95% CI: 0.6% – 1.2%) across 17 studies. In 9 studies with 719 patients, the heart transplant rate was 1.6% (95% CI: 0.6% – 3.9%). Conclusion In this first systematic review and meta-analysis of ICDs in pediatric patients, ICD therapy was effective for prevention of SCD with a 7.0% annualized rate of appropriate ICD shocks. This is comparable to the annualized rate of appropriate ICD shock for secondary prevention of SCD in adult randomized controlled trials. Most common adverse events in the pediatric population were lead failure and system revision. Further refinements in patient selection criteria for implantation and improvements in lead and device technology are needed for pediatric patients.FigureTable