title: Hemophilia A in cardiac operations: a model of reduced thrombin generation.
abstract: Patients with hemophilia A have a congenital defect in thrombin generation. Only limited data are available on the substitution regimens in hemophilia A patients during and after cardiac operations. There are no data on heparinization of these patients during cardiopulmonary bypass. Whereas most case reports suggest factor VIII replacement in combination with standard heparinization to achieve near-normal physiologic factor VIII activity, we describe the successful management of a hemophilic A patient using a low-level factor VIII replacement combined with a reduced heparin dosage during cardiopulmonary bypass. This approach facilitated adequate anticoagulation and minimized the amount of factor VIII treatment necessary without compromising bleeding control.
authors.affiliation: Department of Transfusion and Hemostaseology, Clinic of Anesthesiology, Ludwig Maximilians University of Munich, Munich, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Lison
authors.forename: Michael
authors.initials: M
authors.lastname: Spannagl
authors.forename: Wulf
authors.initials: W
authors.lastname: Dietrich
mesh_terms: D000368:Aged; D000925:Anticoagulants / Q000008:administration & dosage*; D001780:Blood Coagulation Tests; D002315:Cardiopulmonary Bypass / Q000379:methods*; D001026:Coronary Artery Bypass / Q000379:methods*; D023921:Coronary Stenosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D005169:Factor VIII / Q000032:analysis; D005500:Follow-Up Studies; D006467:Hemophilia A / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006493:Heparin / Q000008:administration & dosage; D006801:Humans; D008297:Male; D016343:Monitoring, Intraoperative / Q000379:methods; D011182:Postoperative Care / Q000379:methods; D011859:Radiography; D018570:Risk Assessment; D013917:Thrombin / Q000032:analysis; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D005169:Factor VIII; D006493:Heparin; D013917:Thrombin