Jansen W, Hombach V, Osterspey A, Lübbing H, Arnold G, Behrenbeck D W, Tauchert M
Z Kardiol. 1982 May;71(5):357-64.
Reports on aortic and mitral valve alterations in patients with osteogenesis imperfecta, a congenital connective tissue disorder, are sparse in the literature. Aortic valve incompetence is more common than mitral valve dysfunction. Though the clinico-pathological and histological features of valve alterations are very similar to those seen in Marfan's Syndrome, valve dysfunctions in osteogenesis imperfecta are rarer than in Marfan's Syndrome. In a 23-year-old patient with typical clinical feature of osteogenesis imperfecta, mitral incompetence was detected by echo- and angiography. Patient was provided with a St.-Jude-Medica prosthesis and postoperative course was without any complications.
成骨不全是一种先天性结缔组织疾病,关于该疾病患者主动脉瓣和二尖瓣病变的文献报道较少。主动脉瓣关闭不全比二尖瓣功能障碍更为常见。尽管瓣膜病变的临床病理和组织学特征与马凡综合征所见非常相似,但成骨不全患者的瓣膜功能障碍比马凡综合征更为罕见。在一名具有典型成骨不全临床特征的23岁患者中,通过超声心动图和血管造影检测到二尖瓣关闭不全。患者接受了圣犹达医疗公司的人工瓣膜置换,术后过程无任何并发症。