Congenital valvular aortic stenosis: limited progression during childhood

• Published in: Archives of disease in childhood Vol. 94; no. 7; pp. 531 - 535
• Main Authors: Ten Harkel, A D J, Berkhout, M, Hop, W C, Witsenburg, M, Helbing, W A
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 01-07-2009; BMJ Publishing Group; BMJ Publishing Group Ltd; BMJ Publishing Group LTD
• Subjects: Adolescent; Age; Age Factors; Aortic valve stenosis; Aortic Valve Stenosis - congenital; Aortic Valve Stenosis - physiopathology; Aortic Valve Stenosis - surgery; Attrition (Research Studies); Biological and medical sciences; Cardiology. Vascular system; Child; Child, Preschool; Cohort Studies; Congenital heart defects; Congenital heart disease; Demographic aspects; Development and progression; Diagnosis; Disease Progression; Echocardiography; Electrocardiography; Endocardial and cardiac valvular diseases; Female; General aspects; Heart; Heart Defects, Congenital - physiopathology; Heart Defects, Congenital - surgery; Heart failure; Humans; Infant; Infant, Newborn; Intervention; Male; Measurement Techniques; Medical sciences; Miscellaneous; Morphology; Mortality; Neonates; Netherlands; Patients; Prevention and actions; Prognosis; Public health. Hygiene; Public health. Hygiene-occupational medicine; Reoperation - statistics & numerical data; Retrospective Studies; Risk Factors; Statistical Analysis; Statistical Significance; Studies; Survival; Systole - physiology; Treatment Outcome; Variance analysis; Velocity; Ventricular Dysfunction, Left - physiopathology; Young Children; Netherlands; Human; Pediatrics; Aortic stenosis; Prognosis; Congenital; Cardiac valvular disease; Cardiovascular disease; Evolution; Limit; Child; Congenital disease; Public health
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/adc.2007.134924

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.