Document Detail

Congenital valvular aortic stenosis: limited progression during childhood.
MedLine Citation:
PMID:  19307195     Owner:  NLM     Status:  MEDLINE    
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.
A D J Ten Harkel; M Berkhout; W C Hop; M Witsenburg; W A Helbing
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Publication Detail:
Type:  Journal Article     Date:  2009-03-22
Journal Detail:
Title:  Archives of disease in childhood     Volume:  94     ISSN:  1468-2044     ISO Abbreviation:  Arch. Dis. Child.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-06-22     Completed Date:  2009-09-01     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372434     Medline TA:  Arch Dis Child     Country:  England    
Other Details:
Languages:  eng     Pagination:  531-5     Citation Subset:  AIM; IM    
Department of Pediatric Cardiology, Erasmus MC-Sophia, Rotterdam, The Netherlands.
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MeSH Terms
Age Factors
Aortic Valve Stenosis / congenital,  physiopathology*,  surgery
Child, Preschool
Cohort Studies
Disease Progression
Heart Defects, Congenital / physiopathology*,  surgery
Infant, Newborn
Reoperation / statistics & numerical data
Retrospective Studies
Risk Factors
Systole / physiology
Treatment Outcome
Ventricular Dysfunction, Left / physiopathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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