Aortic and Mitral Valve Replacement in a Patient with Osteogenesis I mperf ecta *

We report a patient with severe aortic and mitral regurgi-tation secondary to osteogenesis imperfecta who success-fully underwent double valve replacement in spite of the collagen defect and a bleeding diathesis. () steogenesis imperfecta is a generalized disorder of connective tissue which causes fragile bones, blue sclerae, hyperextensible joints, conduction deafness and abnormalities of dentition. It is usually transmitted as an autosomal dominant with variable penetrance. In its most severe form, death in utero results from multiple fractures. On the other hand, the only abnormality may be blue sclerae. ’ Aortic and mitral regurgitation secon-dary to the tissue defect can occur in patients who survive to adulthood. We report a patient with severe aortic and mitral regurgitation due to osteogenesis im