Symptomatic congenital aortic stenosis in the first year of life+

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A clinical review of 18 cases of congenital aortic valvular stenosis symptomatic in thefirst year of life was undertaken. The electrocardiogram and thoracic roentgenograms mimic primary endocardial fibroelastosis; auscultatory findings, however, are usually sufficiently localized as to suggest aortic stenosis. Mitral insufficiency was a common finding in left ventriculograms. Pathologic study suggests that infants with aortic stenosis who are symptomatic in the first year of life commonly exhibit infarction of the left ventricular papillary muscles. This, in turn, is a fundamental cause of mitral regurgitation, a factor which may contribute to congestive cardiac failure.

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    Citation Excerpt :

    While not unique to BAV disease, myocardial fibrosis can be seen in children with significant aortic stenosis and is partially reversible after relief of the obstruction (54,55). Children who present with aortic stenosis in infancy have more severe disease and poor outcomes (56–58). Because there is often very little calcification during childhood, balloon valvuloplasty is the treatment of choice for severe aortic stenosis in this age cohort.

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This study was supported by Public HealthService Research Grant HE-5694 and Research Training Grant 5 T1 HE 5570 of the National Heart Institute.

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Address, Department of Pathology, The Charles T. Miller Hospital, 125 West College Avenue, St. Paul, Minn. 55102.

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