Related Experiment Video
Updated: Jun 24, 2026
A Rodent Model of The Ross Operation: Syngeneic Pulmonary Artery Graft Implantation in A Systemic Position
Published on: April 1, 2022
Congenital valvular aortic stenosis: limited progression during childhood
A D J Ten Harkel1, M Berkhout, W C Hop
1Department of Pediatric Cardiology, Erasmus MC-Sophia, Rotterdam, The Netherlands. A.D.J.Ten_Harkel@lumc.nl
Insights
Congenital valvular aortic stenosis in children shows slow progression, but many require intervention. Prognosis is good after infancy, with low mortality rates. Keywords: congenital valvular aortic stenosis, pediatric, intervention, prognosis.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Echocardiography
Background:
- Congenital valvular aortic stenosis is a frequent congenital heart defect.
- The progression rate of this condition during childhood is not well-established.
- Understanding progression is crucial for timely intervention and management.
Purpose of the Study:
- To assess the progression of peak aortic velocity in pediatric patients with isolated congenital valvular aortic stenosis.
- To determine the frequency of cardiac intervention required for this condition.
- To evaluate the long-term prognosis and survival rates based on age at diagnosis.
Main Methods:
- Retrospective cohort study of 245 consecutive pediatric patients.
- Analysis of clinical and echocardiographic data.
- Longitudinal follow-up to track velocity changes and intervention needs.
Main Results:
- Mean annual increase in peak systolic velocity was 0.04 m/s/year.
- 73 patients (30%) required cardiac intervention, more often in younger patients with higher initial peak velocity.
- Mortality was significant in infants (5-year survival 73%) but minimal in older children.
Conclusions:
- Congenital valvular aortic stenosis generally has a favorable prognosis in children beyond the neonatal period.
- Progression is typically limited, but early intervention is often necessary.
- Survival rates are high for patients diagnosed after infancy.
Objective:
Congenital valvular aortic stenosis is a common congenital heart malformation. The rate of progression in childhood, however, remains to be established. We assessed the progression of peak aortic velocity before intervention as well as the frequency of intervention in paediatric patients with isolated congenital valvular aortic stenosis.
Methods:
A retrospective cohort study was performed in 245 consecutive patients with aortic stenosis. Both clinical and echocardiographic data were obtained.
Results:
Over a period of 9.0 (SD 5.2) years (range 0.1-19.4), the mean annual increase in peak systolic velocity was 0.04 m/s/year (95% CI 0.028 to 0.056 m/s/year; p<0.001) as shown by ANOVA. 40 patients underwent a cardiac intervention shortly after their first echocardiogram. Another 33 patients underwent intervention during follow-up. Interventions were performed significantly more often in patients diagnosed at a younger age and/or with a higher peak velocity at diagnosis (p<0.001). Mortality was considerable in those diagnosed in infancy (5-year survival rate of 73% (SD 9%), whereas it was nearly absent in patients diagnosed after infancy. Most patients who died during infancy had progressive left ventricular dysfunction despite adequate relief of left ventricular outflow obstruction.
Conclusions:
Valvular aortic stenosis in the paediatric age group usually has a good prognosis beyond the neonatal period. Progression over time is usually limited, although a considerable proportion of patients need intervention shortly after initial diagnosis. Mortality, except for the neonatal age group, is nearly absent.
Related Concept Videos
01:22Mitral Stenosis I: Introduction
01:26Mitral Stenosis III: Medical Management
01:15Aortic Regurgitation I: Introduction
01:23Mitral Stenosis II: Clinical features and Diagnostic Tests
01:22Aortic Regurgitation II: Clinical Features and Diagnostic Tests
01:25Aortic Regurgitation III: Medical Management