Valvular and aortic diseases in osteogenesis imperfecta

Lamanna, Arvin, Fayers, Trevor, Clarke, Sophie and Parsonage, William (2013) Valvular and aortic diseases in osteogenesis imperfecta. Heart Lung and Circulation, 22 10: 801-810. doi:10.1016/j.hlc.2013.05.640

Author Lamanna, Arvin
Fayers, Trevor
Clarke, Sophie
Parsonage, William
Title Valvular and aortic diseases in osteogenesis imperfecta
Journal name Heart Lung and Circulation   Check publisher's open access policy
ISSN 1443-9506
Publication date 2013-10-01
Year available 2013
Sub-type Article (original research)
DOI 10.1016/j.hlc.2013.05.640
Open Access Status
Volume 22
Issue 10
Start page 801
End page 810
Total pages 10
Place of publication Chatswood, NSW, Australia
Publisher Elsevier
Language eng
Subject 2705 Cardiology and Cardiovascular Medicine
2740 Pulmonary and Respiratory Medicine
Abstract 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 OI 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.
Keyword Osteogenesis imperfecta
Valvular disease
Aortic disease
Valve replacement
Valve repair
Q-Index Code CX
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
ERA White List Items
School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 4 times in Thomson Reuters Web of Science Article | Citations
Scopus Citation Count Cited 8 times in Scopus Article | Citations
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