期刊
HEART LUNG AND CIRCULATION
卷 22, 期 10, 页码 801-810出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.hlc.2013.05.640
关键词
Osteogenesis imperfecta; Valvular disease; Aortic disease; Valve replacement; Valve repair
Osteogenesis imperfecta (OI) is an inheritable connective tissue disorder caused by defective collagen synthesis with the principal manifestations of bone fragility. OI has been associated with left sided valvular regurgitation and aortic dilation. Valve and aortic surgery are technically feasible in patients with OI but are inherently high risk due to the underlying connective tissue defect. This report reviews the valvular and aortic pathology associated with OI and their management. We describe two cases of patients with CH who have significant aortic and mitral valve regurgitation, one of whom has been managed conservatively and the other who has undergone successful mitral valve repair and aortic valve replacement. The latter case represents the fifth case of mitral valve repair in a patient with OI reported in the medical literature. Crown Copyright (C) 2013 Published by Elsevier Inc. on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.
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