Congenital aortic valvar stenosis: limited progression during childhood

Arend Derk Jan Ten Harkel ^1*, Maarten Berkhout ¹, Wim C Hop ¹, Maarten Witsenburg ¹ and Willem A Helbing ¹

¹ Erasmus MC-Sophia, Netherlands

^* To whom correspondence should be addressed. E-mail: a.d.j.ten_harkel{at}lumc.nl.

Accepted 17 February 2009

Abstract

Objective: Congenital valvular aortic stenosis is a common congenital heart malformation. The rate of progression in childhood, however, remains to be established. We assessed progression of peak aortic velocity before intervention as well as frequency of intervention in pediatric patients with isolated congenital valvular aortic stenosis.

Patients and 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±5.2 years (range 0.1-19.4) mean annual increase of peak systolic velocity was 0.04 m/sec/year (95% confidence interval 0.028 to 0.056 m/sec/year; P<0.001) as shown by Anova. Forty patients underwent any 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 younger age and/or with a higher peak velocity at diagnosis (P<0.001). Mortality was considerable in those diagnosed in infancy (five-year survival 73±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 LV outflow obstruction.

Conclusions: Valvular aortic stenosis in the pediatric age group usually has a good prognosis beyond neonatal age. Progression over time is usually limited, although a considerable proportion of patients need any intervention shortly after the initial diagnosis. Mortality, except for the neonatal age group, is nearly absent.