Delayed Onset of Hemolytic Anemia in a Child

Severe intravascular hemolysis is described as a complication of implantation of an aortic Starr-Edwards ball-valve prosthesis and insertion of a Teflon patch in the ascending aorta in a 9-year-old boy with severe aortic valvar stenosis. This progressive hemolysis of delayed onset was the sole indicator of extreme degeneration of the silicone rubber ball of the prosthetic aortic valve, a potentially lethal condition requiring surgical intervention. The phonocardiographic signs of aortic ball variance were not present, and there was no overt valvar incompetence. A lesser but noteworthy aspect in this case was the occurrence of an "aplastic crisis" superimposed on the severe hemolysis. This transient bone marrow, red cell aplasia, which was unrelated to drugs, precipitated an acutely severe anemia which accounted for the presenting symptoms of the patient and required transfusion.