• J Extra Corpor Technol · Dec 2008

    Case Reports

    Mitral valve replacement in a patient with sickle cell disease using perioperative exchange transfusion.

    • David Chabot and Robin Sutton.
    • Department of Perfusion Technology, Rush University Medical Center, Chicago, Illinois 60612, USA. David_L_Chabot@rush.edu
    • J Extra Corpor Technol. 2008 Dec 1; 40 (4): 275-7.

    AbstractSickle cell disease is a genetic hemoglobinopathy in which a significant number of red blood cells carry hemoglobin-S as opposed to normal red blood cells that contain hemoglobin-A. Under certain conditions such as hypoxia, acidosis, and hypothermia, the red blood cells containing hemoglobin-S will sickle, leading to occlusion of the microvasculature. As such, patients with sickle cell disease present unique challenges during heart surgery using cardiopulmonary bypass (CPB). After conducting a literature review, we discovered that the exact hemoglobin-S level for conducting cardiac surgery with CPB is not known. However, a hemoglobin-S level < 30% is considered safe for conducting CPB. The following case report will discuss these challenges and present a patient with sickle cell disease undergoing a mitral valve repair. Management of this patient involved exchange transfusions both preoperatively and intraoperatively.

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