Objectives
To support clinical decision-making in children with aortic valve disease, this study aims to compile the available evidence on outcome after paediatric aortic valve repair.
Methods
A systematic review of literature reporting clinical outcome after paediatric aortic valve repair (mean age at surgery <18y) published between 01/01/1990-23/12/2021 was conducted. Early event risks, late event rates and time-to-event data were pooled. A microsimulation model was employed to simulate lives of children.
Results
Forty-one publications were included, encompassing 2,623 patients with 17,217 patient-years of follow-up (median follow-up:7.3y; range:1.0-14.4y). Pooled mean age during repair for aortic stenosis in children(<18y), infants(<1y) or neonates(<30d) 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 2.7%(95%CI:1.7%-4.3%), 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 4.19%/y(2.94-5.96%/y), 6.84%/y(3.95-11.83%/y), and 6.32%/y(3.04-13.15%/y), endocarditis 0.25%/y(0.09-0.70%/y), 0.23%/y(0.07-0.71%/y), and 0.49%/y(0.18-1.29%/y), and valve thrombosis 0.14%/y(0.05-0.34%/y), 0.15%/y(0.04-0.53%/y), and 0.19%/y(0.05-0.77%/y). Microsimulation-based mean life-expectancy in the first 20 years for children, infants and neonates with aortic stenosis, respectively, was 18.8y(95%CrI:18.6-18.9y; relative survival compared to general population: 94.2%), 16.8y(16.5-17.0y; relative survival: 84.2%) and 15.9y(14.8-17.0y; 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
Outcome after paediatric aortic valve repair for stenosis is dependent on age at surgery. Despite a high hazard of reintervention for valve dysfunction and slightly impaired survival relative to the general population, aortic valve repair is associated with low valve-related event occurrences.
PROSPERO
CRD42022292320