Aortic valve repair in neonates, infants and children: a systematic review, meta-analysis and microsimulation study

OBJECTIVES: To support clinical decision-making in children with aortic valve disease, by compiling the available evidence on outcome after paediatric aortic valve repair (AVr). METHODS: A systematic review of literature reporting clinical outcome after paediatric AVr (mean age at surgery <18 years) published between 1 January 1990 and 23 December 2021 was conducted. Early event risks, late event rates and time-to-event data were pooled. A microsimulation model was employed to simulate the lives of individual children, infants and neonates following AVr. RESULTS: Forty-one publications were included, encompassing 2 623 patients with 17 217 patient-years of follow-up (median follow-up: 7.3 years; range: 1.0–14.4 years). Pooled mean age during repair for aortic stenosis in children (<18 years), infants (<1 year) or neonates (<30 days) was 5.2 ± 3.9 years, 35 ± 137 days and 11 ± 6 days, respectively. Pooled early mortality after stenosis repair in children, infants and neonates, respectively, was 3.5% (95% confidence interval: 1.9–6.5%), 7.4% (4.2–13.0%) and 10.7% (6.8–16.9%). Pooled late reintervention rate after stenosis repair in children, infants and neonates, respectively, was 3.31%/year (1.66–6.63%/year), 6.84%/year (3.95–11.83%/year) and 6.32%/year (3.04–13.15%/year); endocarditis 0.07%/year (0.03–0.21%/year), 0.23%/year (0.07–0.71%/year) and 0.49%/year (0.18–1.29%/year); and valve thrombosis 0.05%/year (0.01–0.26%/year), 0.15%/year (0.04–0.53%/year) and 0.19%/year (0.05–0.77%/year). Microsimulation-based mean life expectancy in the first 20 years for children, infants and neonates with aortic stenosis, respectively, was 18.4 years (95% credible interval: 18.1–18.7 years; relative survival compared to the matched general population: 92.2%), 16.8 years (16.5–17.0 years; relative survival: 84.2%) and 15.9 years (14.8–17.0 years; relative survival: 80.1%). Microsimulation-based 20-year risk of reintervention in children, infants and neonates, respectively, was 75.2% (72.9–77.2%), 53.8% (51.9–55.7%) and 50.8% (47.0–57.6%). CONCLUSIONS: Long-term outcomes after paediatric AVr for stenosis are satisfactory and dependent on age at surgery. Despite a high hazard of reintervention for valve dysfunction and slightly impaired survival relative to the general population, AVr is associated with low valve-related event occurrences and should be considered in children with aortic valve disease.