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Korean Journal of Anesthesiology 2008;55(2):233-236.
DOI: https://doi.org/10.4097/kjae.2008.55.2.233   
The anesthetic management of mitral valve replacement in the patient with paroxysmal nocturnal hemoglobinuria: A case report.
Sung Jin Hong, Ji Young Lee, Jin Young Chon, Jung Hyun Park
Department of Anesthesia and Pain Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea. hongs@catholic.ac.kr
Paroxysmal nocturnal hemoglobinuria (PNH) is a disorder of stem cells resulting in complement-mediated red cell lysis. Potential complications of cardiac surgery in patients with PNH include increased risk of infection due to neutropenia, hemolytic crisis as a result of complement activation during cardiopulmonary bypass, and bleeding if thrombocytopenic. We report a case of anesthetic management for mitral valve replacement surgery in a patient with PNH. Preoperative management included the administration of granulocyte-stimulating factor, red cell transfusion, steroid, and prophylactic antibiotics and prevention of thrombosis. Intraoperative and early postoperative management included filtering leukocyte from homologous blood, avoidance of plasma transfusion and replacement of platelet to control bleeding. Although the postoperative course was not smooth because of delayed wound healing, the patient had recovered well.
Key Words: cardiac surgery; paroxysmal nocturnal hemoglobinuria


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