Intravascular Hemolytic Syndrome Following Aortic Valve Replacement:  Complete Hematologic and Cardiac Recovery Without Surgical Intervention

Arch Intern Med. 1966;118(5):486-490. doi:10.1001/archinte.1966.00290170074013.
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INTRAVASCULAR hemolytic anemia and bizarre red blood cell fragmentation has been an infrequent but spectacular complication of open heart surgery. More specifically, this syndrome has occurred in association with defective intracardiac prosthetic implants, both interatrial patch grafts 1-7 and valve prostheses.7-16

Of the 15 patients with well-documented "traumatic" hemolytic anemia following aortic valve replacement, only two instances of complete hematologic recovery have been noted, both of which attended replacement of the defective prosthesis.8 Excluding five deaths, the remaining eight patients all showed evidence of persistent hemolytic anemia or a compensated hemolytic state, ie, reticulocytosis, red blood cell (RBC) fragmentation, and a shortened RBC life span (Table).

We have recently studied a patient who developed intravascular hemolytic anemia following implantation of an aortic cagedball valve prosthesis. Although the hematologic features of his illness were similar to previously reported cases, the ultimate course of the hemolytic process was unique in that


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