title: Mitral valve replacement in a patient with sickle cell disease using perioperative exchange transfusion.
abstract: Sickle 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.
authors.affiliation: Department of Perfusion Technology, Rush University Medical Center, Chicago, Illinois 60612, USA. David_L_Chabot@rush.edu
authors.forename: David
authors.initials: D
authors.lastname: Chabot
authors.forename: Robin
authors.initials: R
authors.lastname: Sutton
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000503:physiopathology*; D002315:Cardiopulmonary Bypass / Q000295:instrumentation* / Q000379:methods; D005078:Exchange Transfusion, Whole Blood / Q000379:methods*; D005260:Female; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006451:Hemoglobin, Sickle / Q000032:analysis*; D006801:Humans; D008943:Mitral Valve / Q000601:surgery*; D019990:Perioperative Care; D012307:Risk Factors; D014914:Whole Blood Coagulation Time
chemical_list: D006451:Hemoglobin, Sickle