title: Mitral valve reconstruction in sickle cell disease.
abstract: As survival improves in patients with sickle cell anemia, the prospects of performing cardiac surgical procedures on older patients with this genetic defect increase. We describe the successful management of a 52-year-old patient with sickle cell disease (homozygous for hemoglobin S) and a history of multiple sickle crisis undergoing cardiopulmonary bypass for mitral valve repair. Preoperative partial exchange transfusion followed by total exchange transfusion at the time of operation was performed to reduce the level of hemoglobin S to 5.4% during bypass. Other management strategies included high-flow normothermic bypass with aortic crossclamping, topical hypothermia, and cold crystalloid cardioplegia.
authors.affiliation: Department of Surgery, University of Michigan Hospitals, Ann Arbor 48109, USA.
authors.forename: F D
authors.initials: FD
authors.lastname: Pagani
authors.forename: R J
authors.initials: RJ
authors.lastname: Polito
authors.forename: S F
authors.initials: SF
authors.lastname: Bolling
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D002314:Cardioplegic Solutions / Q000008:administration & dosage; D002315:Cardiopulmonary Bypass / Q000379:methods; D003080:Cold Temperature; D000077324:Crystalloid Solutions; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D006324:Heart Arrest, Induced; D006451:Hemoglobin, Sickle / Q000032:analysis / Q000235:genetics; D006579:Heterozygote; D006801:Humans; D007036:Hypothermia, Induced; D007552:Isotonic Solutions; D008875:Middle Aged; D008943:Mitral Valve / Q000601:surgery*; D008944:Mitral Valve Insufficiency / Q000601:surgery*; D010952:Plasma Substitutes / Q000008:administration & dosage
chemical_list: D002314:Cardioplegic Solutions; D000077324:Crystalloid Solutions; D006451:Hemoglobin, Sickle; D007552:Isotonic Solutions; D010952:Plasma Substitutes