[Valve replacement for mitral in incompetence in a patient with osteogenesis imperfecta (author's transl)]

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.