Anaemia, osteogenesis imperfecta and valve diseases. The preoperative treatment with epoetin-alpha to increase haematocrit and haemoglobin levels in patients with high risk of perioperative bleeding.

The case of a patient with Osteogenesis imperfecta is reported who underwent surgery for mitral valve replacement. Osteogenesis imperfecta is a hereditary disease of the connective tissue, associated with bone fragility, bluish colouring of the sclerae, loss of hearing and dental anomalies. Osteogenesis imperfecta is included in a group of hereditary pathologies with Ehlers-Danlos syndrome, Hurler syndrome, pseudoxanthoma elasticum and Marfan syndrome. In the literature there are few cases of patients with osteogenesis imperfecta operated for mitral valve disease secondary to such disease. The patient also has a positive family history and a severe anaemia: this was treated with epoetin-alpha and ferrous sulphate during the three weeks before surgery. The response to epoietin treatment was good while clinical outcome was poor due to rupture of the posterior ventricular wall in the 12th postoperative hour. The use of epoietin-alpha is discussed.