Spontaneous Partial Remission of Postoperative Hemolytic Anemia in a Case with Ostium Primum Defect

SEVERE hemolytic anemia after surgical correction of congenital endocardial cushion defects is a rare clinical entity, first described by Sayed et al. in 1961.1 Since then five more cases have been reported.2 4 All of these patients had an endocardial cushion defect with a cleft in the mitral valve. All defects were repaired with a Teflon patch, and the patients developed hemolytic anemia in the postoperative period. The hemolysis was considered to be mechanical in origin caused by regurgitation of blood through the residual cleft in the mitral valve against the Teflon patch. -4 Only one patient is reported to have demonstrated spontaneous remission although, after 8 months, splenomegaly and elevated reticulocyte count still persisted.2 Sayed's patient had an immediate cessation of hemolysis after a second operation at which the bare Teflon patch was covered with endocardium.1 The remaining four patients were reoperated upon. Of the two patients reported by Sigler et al.2 3 one showed a complete remission and one died on the third postoperative day. Of the two cases reported by Verdon et al.,4 one patient did not show remission and died 6 months after the second operation from massive pulmonary embolism. The other patient died 6 hours after the second operation.