[Aortic regurgitation with osteogenesis imperfecta].

A 49-year-old male was referred to our hospital for cardiomegaly and severe aortic regurgitation. He had been diagnosed with osteogenesis imperfecta (OI) due to his history of multiple fractures in childhood and blue sclera. Aortic valve replacement(AVR) was performed via femoral cannulation and full sternotomy, with the opening of the sternum limited to a width of 6-7 cm in order to protect the brittle sternum and thorax. After weaning the patient off cardiopulmonary bypass, the sternum was closed using titanium plates. He subsequently recovered without excessive bleeding or other complications, and the healing of the sternum 3 months after the surgery was satisfactory. Cardiovascular surgery has been reported to be associated with high morbidity and mortality in patients with OI due to their friability of tissue and bleeding tendency. In the present case, AVR was performed successfully in a patient with OI using various surgical techniques.