title: Sickle cell disease and aortic valve replacement: use of cardiopulmonary bypass, partial exchange transfusion, platelet sequestration, and continuous hemofiltration.
abstract: Sickle cell disease in patients undergoing open heart procedures presents a multitude of challenges to the medical staff. With improved techniques of cardiopulmonary bypass, surgery, and anesthesia for treating patients with sickle cell disease, perfusionists will likely encounter patients with this genetic disorder on a more frequent basis. A 40-year-old black woman was admitted to our institution with recurrent Staphylococcus epidermidis and sepsis. She underwent transesophageal echocardiography and cardiac catheterization and was subsequently diagnosed with severe aortic insufficiency. The aortic valve was replaced. Herein, we report our experience in the preoperative, perioperative, and postoperative management of this patient. We present a concise update on the current literature and techniques used by others in similar cases, and we provide a brief section on future considerations to assist fellow practitioners in recognizing this disease and meeting the accompanying challenges.
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Baylor University Medical Center, Dallas, Texas 75246, USA.
authors.forename: S W
authors.initials: SW
authors.lastname: Sutton
authors.forename: E K
authors.initials: EK
authors.lastname: Hunley
authors.forename: M A
authors.initials: MA
authors.lastname: Duncan
authors.forename: R
authors.initials: R
authors.lastname: Rodriguez
authors.forename: T P
authors.initials: TP
authors.lastname: Meyers
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001021:Aortic Valve / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000150:complications* / Q000601:surgery*; D002315:Cardiopulmonary Bypass; D005078:Exchange Transfusion, Whole Blood / Q000379:methods*; D005260:Female; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006440:Hemofiltration / Q000379:methods; D006801:Humans; D017713:Platelet Transfusion / Q000379:methods