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Background: Pediatric aortic root dilatation is a life-threatening condition that lacks guidelines for surgical management. We aimed to analyze the data on aortic valve interventions during root surgery to guide decision-making. Methods: A search was performed of MEDLINE, Embase, CENTRAL, ClinicalTrials.gov , and WHO ICTRP. Citations were screened in duplicate and independently to identify randomized controlled trials, cohorts, and case series involving populations aged 0 to 18 years, who received valve-sparing and valve-replacing aortic root surgeries between 1999 and 2019. Outcomes considered included mortality (perioperative, one year, five year), reintervention rates. Results: After duplicate removal, 689 citations were screened through abstract and full text review, identifying five eligible studies. All five were observational studies evaluating valve-sparing procedures. There were 81 patients with a mean study age range of 9.9 to 13.9 years. Both reimplantation (74%) and remodeling (26%) subtypes were done. Range of mean duration of follow-up was 1.2 to 4.4 years. There was no mortality reported until the one-year follow-up period. The long-term mortality rate was calculated as 0.02 per patient-year (95% CI: 0.01-0.05). The long-term reintervention rate was 0.08 per patient-year (95% CI: 0.05-0.13). Conclusions: There is limited experience on aortic valve intervention during aortic root surgery in children. Single-arm studies on valve-sparing surgeries show excellent survival up to one year. Mortality and reintervention rates increase in the longer term. The small sample size and lack of controlled studies do not allow for direct comparisons between procedure types.

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