[Aortic valve replacement without blood transfusion in a patient with osteogenesis imperfecta]

Osteogenesis imperfecta (OI) is a hereditary systemic connective tissue disorder that is rarely associated with valvular heart diseases. Cardiovascular surgery for patients with OI carries high morbidity and mortality, which mostly results from bleeding tendencies caused by tissue and capillary fragility. We report a 47-year-old male with isolated aortic regurgitation, who underwent aortic valve replacement (AVR) with a mechanical prosthesis without blood transfusion. The perioperative course was uneventful. We believe that a meticulous technique and a planned strategy to avoid every damage to fragile tissues, particularly to bones, are very important during surgery for patients with OI.