Aortic and Mitral Valve Replacement in a Patient with Severe Haemophilia A

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Abstract
A 49 yr old man with severe hemophilia A developed left ventricular failure as a complication of valvular heart disease. On Nov. 26, 1979, open heart surgery was performed and the aortic and mitral valves were replaced with porcine heterografts. Replacement therapy in the form of Factor VIII concentrates maintained the Factor VIII coagulant level at 100% until 10 days postoperatively and a total dose of 116,600 U were given. The patient described is the 1st hemophiliac to undergo open heart surgery in Australia and the 1st reported case in the world to have a successful double valve graft. With the current availability of factor VIII concentrates, elective surgical procedures are being performed more frequently. Such procedures require careful preoperative planning and postoperative monitoring of replacement therapy to ensure satisfactory hemostasis. Although hemostatic defects may occur after open heart surgery, this case report illustrates that the hemostatic problems of open heart surgery and hemophilia can be prevented by preoperative planning and meticulous postoperative monitoring of replacement therapy.

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