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Perioperative Management of Hemophilia A Patients Undergoing Cardiac Surgery: A Literature Review of Published Cases

      Objective: To investigate the perioperative management of patients with hemophilia A (HA) who undergo cardiac surgery.

      Design

      Retrospective analysis of the published literature.

      Setting

      University hospital.

      Participants

      HA patients undergoing cardiac surgery.

      Interventions

      The PubMed, Embase, Web of Science Core Collection, CNKI, VIP, and WANFANG databases were searched with the terms “hemophilia A,” “cardiac surgery,” “cardiopulmonary bypass,” “coronary artery bypass graft,” “valve disease,” and “congenital heart disease” to identify relevant articles. Data regarding patient characteristics, perioperative management protocols, and clinical outcomes were extracted and analyzed.

      Measurements and Main Results

      Fifty articles, with 72 patients aging from 12 days to 80 years, were included. The analyzed population consisted of 36 (50%) mild HA, 10 (14%) moderate HA, and 25 (35%) severe HA patients. Factor VIII inhibitors were detected in 7 cases. Surgical procedures included 27 coronary artery bypass grafting procedures, 15 valve surgeries, 8 combined coronary artery bypass grafting with valve surgery procedures (1 with left ventricular assist device), 1 ventricular aneurysm resection with valve surgery, 11 corrections of congenital heart disease, 7 adult aortic surgeries, 3 heart transplantations, and 1 repair of heart injury. Factor VIII concentrates were used as a substitution therapy to ensure normalization of perioperative coagulation function. Although an uneventful clinical outcome was achieved in the majority of cases, 20% of patients developed complications with different severities.

      Conclusions

      Execution of a perioperative management strategy with a multidisciplinary approach, a thorough factor replacement protocol, and careful monitoring of factor levels facilitate an optimal outcome for HA patients undergoing cardiac surgery.

      Key Words

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