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.

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A 45-year-old female with Marfan syndrome had a Bentall's procedure performed 19 years ago. She presented with a 4-year history of gradually worsening dyspnea and decreasing exercise tolerance. Investigations revealed severe mitral valve prolapse, a left main stem coronary artery (LMSCA) aneurysm, and a recurrent aneurysm of the ascending aorta. The mitral valve was replaced and the aortic aneurysmal sac and the LMSCA aneurysm were then repaired by a modified Bentall procedure. The patient made an uneventful recovery and was discharged home.