Bentall Operation in a Patient With Severe Hemophilia A and Marfan Syndrome by Use of a Biologic Composite Graft
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We describe a patient with severe hemophilia A and Marfan syndrome who underwent an elective Bentall operation. Because of the severe hemophilia, anticoagulation could not be given postoperatively; thus, a biologic Valsalva conduit graft was used. During the procedure, factor VIII was given as a bolus dose just before incision, then by continous infusion intra-operatively to maintain the factor VIII activity level between 200% and 300%. Minimal postoperative bleeding occurred. The infusion was continued postoperatively at a lower dose until all chest tubes, pacing wires, and invasive catheters were removed. The patient was discharged on postoperative day 7 without adverse events. (C) 2016 by The Society of Thoracic Surgeons