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AIM: Osteogenesis imperfecta (OI) is an inherited connective tissue disorder in which fragile bones readily cause fractures. Aortic root dilatation, aortic valve regurgitation and mitral valve prolapse are uncommon cardiovascular manifestations of OI. Cardiac surgery in these patients carries a high risk of complications due to increased tissue and capillary fragility. We describe an open heart surgery in a woman with isolated aortic valve regurgitation secondary to OI. MATERIAL OF STUDY: A 58-year-old woman was referred to our hospital for surgical correction of aortic valve regurgitation. She had a past history of recurrent long bone fractures, and OI was diagnosed in the childhood. A standard median sternotomy was performed; the sternum was found to be thin and brittle. The native aortic valve was replaced with a size 23 mm stented aortic bioprosthesis. The sternum was closed with stainless steel wires. RESULTS: The postoperative course was uneventful, and the patient was discharged home on the eighth postoperative day. We used thoracic band to avoid sternal diastasis. One year postoperatively, the echocardiogram showed a normal aortic bioprosthesis function without paravalvular leakage. The sternum was stable without dehiscence. DISCUSSION:The mortality rate in cardiac surgery patients with heritable generalized connective tissue disorders, such as osteogenesis imperfecta, is high. Although tissue friability had no impact on surgical outcome, it should be kept in mind when operating on patients with OI. CONCLUSIONS: We highlight the importance of a meticulous surgical technique, together with a strategy for management of anticipated perioperative complications to ensure a successful outcome.