Intractable hemolysis caused by perivalvular leakage following mitral valve replacement with St. Jude Medical prosthesis.

Nine patients with intractable hemolysis caused by perivalvular leakage following mitral valve replacement with a St. Jude Medical prosthesis are presented. All patients had dark-colored hemoglobinuria, which appeared from 1 day to 44 days after the operation, with moderate or severe hepatorenal insufficiency. One patient died of multiorgan failure. The other 8 patients underwent reoperation, and all survived. Reoperation revealed that all leakages were tiny and had no adverse effect on hemodynamics. In all the patients having reoperation, hemoglobinuria disappeared immediately after the procedure. Surface-scanning electron microscopy of the sewing cuff of the St. Jude Medical prosthesis revealed the rough surface structure of the sewing ring. Because of this irregular, shaggy surface structure, greater shearing forces against erythrocytes can be generated when there is perivalvular leakage.