Hemolytic Anemia Due to a Defective Teflon Aortic Valve Prosthesis

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Abstract
Severe intravascular hemolysis following cardiac surgical procedures has now been reported in a number of patients. The case reported here is thought to represent the first of this type of anemia resulting from a defective Teflon aortic valve prosthesis. This patient developed severe anemia, jaundice and low grade fever a year after the replacement of her aortic valve with Teflon leaflets. Studies demonstrated an intravascular hemolytic process with marked fragmentation of the erythrocytes as observed on the peripheral blood smear. The hemolysis was not corrected by corticosteriods, splenectomy or immuno-suppressive therapy (azothioprine). The diagnosis of a mechanical hemolytic anemia was made on the basis of the history and the bizarre red cell morphology. Unfortunately she expired before replacement of the offending Teflon leaflets could be achieved. On post mortem examination, the prosthetic leaflets were found to be rigid, calcified and unendothelialized with a tear through the non-coronary cusp. The mechanisms by which the prosthesis could have caused red blood cell destruction are discussed. The important factors may include one or a combination of the following: the chemical effect of the prosthesis on the erythrocytes, the roughness of the prosthetic surface, the force of impact of the red blood cells against the prosthesis, turbulent flow produced by the defective prosthesis. This syndrome has not been seen frequently. However, with the increasing use of various cardiac prostheses and the longer survival of patients with these devices, the incidence of this type of anemia may increase. Awareness of this entity may prove life saving since the only possible therapy, if hemolysis is severe, is surgical correction of the defect.

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