Aortic valve replacement in osteogenesis imperfecta--technical and practical considerations for a successful outcome.

Osteogenesis imperfecta is a connective tissue disorder that is rarely associated with isolated aortic insufficiency. Surgery on such patients carries high morbidity and mortality, which are mostly associated with bleeding tendencies secondary to increased tissue and capillary fragility. We report a 42-year-old male with isolated aortic incompetence, who underwent aortic valve replacement (AVR) with a mechanical prosthesis. The postoperative course was uneventful, and the patient was discharged home on the seventh postoperative day (POD 7). He remains well on follow-up 6 months later. We highlight the importance of a meticulous surgical technique, together with a strategy for management of anticipated perioperative complications to ensure a successful outcome.