The significance of discontinuity between the aortic and mitral valves in the presence of "normally related" arterial trunks.
We describe a case in which an extensive sub-aortic infundibulum was accompanied by a muscular ventricular defect in the presence of a normal relationship of the great arteries. First impressions suggested that the ventriculo-arterial connexion was concordant, but sectioning the heart showed it to be double outlet from the right ventricle. It is important to identify this combination of features prior to attempted surgical correction, since the potentially restrictive sub-aortic infundibulum may not be visualised if the heart is approached surgically from the right ventricle. If unrelieved in the repair process, this could leave severe sub-aortic stenosis.
Ruchelli
E D
ED
Department of Pathology, Children's Hospital of Pittsburgh, Pennsylvania.
Anderson
R H
RH
D001021:Aortic Valve / Q000002:abnormalities* / Q000473:pathology; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000473:pathology*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008943:Mitral Valve / Q000002:abnormalities* / Q000473:pathology