Successful aortic valve replacement in osteogenesis imperfecta: with special emphasis on peri-operative management.

Valvular heart disease is a recognized feature of the connective tissue diseases. It occurs to a variable extent in osteogenesis imperfecta and frequently involves the aortic valve. Replacement of the aortic valve, although required, may be complicated by bleeding problems secondary to platelet dysfunction and capillary fragility. A successful aortic valve replacement in a patient with type I osteogenesis imperfecta is described with special reference to hematologic manipulations to control bleeding post-operatively.