1 From the Departments of Pediatrics and Surgery, University of Florida College of Medicine, Gainesville, Florida, and the Medical Center Clinic, Pensacola, Florida.
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.
© 1969 American Heart Association, Inc.
Delayed Onset of Hemolytic Anemia in a Child
An Indicator of Ball Variance of Aortic Valve Prosthesis
Key Words: Cardiac intravascular hemolysis • Aplastic crisis • Silicone rubber ball degeneration • Starr-Edwards aortic valve prosthesis
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  M. Stefanini, J. S. Curtis, and J. J. Walsh Intravascular Hemolysis and Consumption Coagulop- Athy in Defective Aortic Valve Prosthesis. Transient, Beneficial Effect of Heparin and Recovery Following Successful Replacement of Valve Vascular and Endovascular Surgery, March 1, 1972; 6(2): 69 - 78. [PDF]
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