Cardiac manifestations in osteogenesis imperfecta. A case report and therapeutic implications

Osteogenesis imperfecta is an hereditary defect in the synthesis of collagen fibres. The disease can cause cardiac defects, such as aortic and mitral incompetence. Surgical repair of these defects is complicated by tissue friability and bleeding problems. We describe a case where an incompetent aortic valve was successfully replaced by a mechanical prosthesis.