Bentall Operation in a Patient with Severe Hemophilia A and Marfan Syndrome by Use of a Biologic Composite Graft
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Date
2016
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Abstract
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 intraoperatively 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. © 2016 The Society of Thoracic Surgeons.
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Adult , Aortography , Biological Products , Blood Vessel Prosthesis , Cardiovascular Surgical Procedures , Elective Surgical Procedures , Follow-Up Studies , Heart Valve Prosthesis , Hemophilia A , Humans , Male , Marfan Syndrome , Prosthesis Design , blood clotting factor 8 concentrate , biological product , adult , anticoagulation , aorta valve regurgitation , aorta valve replacement , Article , ascending aorta surgery , bentall operation , cardiopulmonary bypass , case report , composite graft , composite material , computed tomographic angiography , disease severity , drug dose increase , echocardiography , elective surgery , funnel chest , heart surgery , hemophilia A , human , male , Marfan syndrome , outcome assessment , physical examination , postoperative hemorrhage , postoperative period , priority journal , surgical drainage , surgical technique , treatment planning , aortography , blood vessel prosthesis , cardiovascular surgery , complication , follow up , heart valve prosthesis , hemophilia A , Marfan syndrome , procedures , prosthesis design