CASE HISTORY

This was an incidental finding in a child who died of congenital agranulocytosis. There is no apparent connection between the cause of death and this curious lesion 

PATHOLOGY

Heart 130mmx115x80mm. The heart is of about normal size for a child age 2. It has been opened by cutting through between the wall of the left ventricle and the inter-ventricular septum posteriorly. Looking through this opening one can see a raised, white, fibrous bar that runs obliquely across the upper part of the ventricular septum, about 5-10mm below the cusps of the aortic value.