Dimitrakakis Georgios, Rathod Jitendrakumar, von Oppell Ulrich Otto, Pericleous Agamemnon, Hutchison Stephen
Department of Cardiothoracic Surgery, University Hospital of Wales, Heath Park, Cardiff, United Kingdom.
Cardiovasc J Afr. 2013 Oct 23;24(9-10):e4-7. doi: 10.5830/CVJA-2013-070.
Osteogenesis imperfecta (OI) is a heritable disorder of the connective tissue. Cardiovascular involvement is rare, related mainly to aortic valve regurgitation. Open-heart surgery in these patients is associated with increased morbidity and mortality rates as a result of tissue friability and bone brittleness as well as platelet dysfunction. We present a patient with OI who underwent successful aortic valve replacement with a mini-sternotomy approach.
成骨不全症（OI）是一种结缔组织遗传性疾病。心血管受累较为罕见，主要与主动脉瓣反流有关。由于组织脆弱、骨骼脆性以及血小板功能障碍，这些患者进行心脏直视手术时发病率和死亡率会增加。我们报告了一名采用微创胸骨切开术成功进行主动脉瓣置换的成骨不全症患者。
