Reversed offsetting of the septal attachments of the atrioventricular valves and Ebstein's malformation of the morphologically mitral valve.
Ebstein's anomaly of the tricuspid valve is characterised by downstream displacement of the tricuspid leaflets. In the case of the septal leaflet, the result is exaggerated offsetting of the atrioventricular valves. Thus it might be supposed that in Ebstein's anomaly of the mitral valve, downstream displacement of the aortic (septal) leaflet of the mitral valve could move its attachment beyond that of the tricuspid valve, resulting in reversed offsetting. To examine this hypothesis three patients each with a concordant atrioventricular connection were studied. The first, with Marfan's syndrome and without an abnormal mitral valve, had reversed offsetting shown by echocardiography. The second, without Marfan's syndrome and with Ebstein's anomaly of the mitral valve, showed echocardiographically normal offsetting but a displaced mural leaflet. The third, in whom Ebstein's anomaly of the mitral valve was confirmed at necropsy, showed all the features of the second case, with normal offsetting of the atrioventricular valves. Thus reversed offsetting is not a feature of Ebstein's anomaly of the mitral valve and in the first case this feature was caused by upward displacement of the tricuspid valve secondary to dilation of the aortic root.
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D004437:Ebstein Anomaly / Q000150:complications* / Q000175:diagnosis; D004452:Echocardiography; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis; D014261:Tricuspid Valve / Q000002:abnormalities*