Aortic and mitral valve replacement in a patient with severe haemophilia A

A forty-nine year old man with severe Haemophilia A developed left ventricular failure as a complication of valvular heart disease. On November 26th, 1979, open heart surgery was performed and both 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 ten days post-operatively and a total dose of 116,600 units were given. The patient described is the first haemophiliac to undergo open heart surgery in Australia and the first reported case in the world to have had 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 pre-operative planning and post-operative monitoring of replacement therapy to ensure satisfactory haemostasis. Although haemostatic defects may occur after open heart surgery, this case report illustrates that the haemostatic problems of open heart surgery and haemophilia can be prevented by pre-operative planning and meticulous post-operative monitoring of replacement therapy.