OSTEOGENESIS IMPERFECTA AND CARDIOVASCULAR-DISEASES

Aortic and mitral valvular insufficiency in patients with osteogenesis imperfecta result from an underlying defect in connective tissue form ation. The surgical cases reported in the literature have included mec hanical and bioprosthetic valve replacement as well as attempts at rep air and reconstruction. Despite complications related to bleeding and tissue friability, acceptable results have been obtained. In this repo rt, we describe aortic regurgitation secondary to osteogenesis imperfe cta treated with homograft replacement. The unique cardiovascular comp lications of osteogenesis imperfecta and the available therapeutic opt ions are discussed in light of the literature review.