Elsevier

American Heart Journal

Volume 94, Issue 5, November 1977, Pages 637-641
American Heart Journal

Valvular heart disease in osteogenesis imperfecta

https://doi.org/10.1016/S0002-8703(77)80135-XGet rights and content

Summary

Aortic and mitral valve abnormalities have been reported which clearly appear to be related to the underlying connective tissue disorder in two patients, a father and daughter, with osteogenesis imperfecta. Although this appears to occur with a much lower prevalence and lesser severity than in the Marfan syndrome, the true prevalence of cardiac connective tissue involvement is not known, and the orthopedic complications of osteogenesis imperfecta may have overshadowed attention to cardiovascular abnormalities. In evaluating patients with osteogenesis imperfecta, careful attention should be paid to cardiovascular findings and if valvular lesions are noted, patients should be instructed regarding the need for antibiotic prophylaxis for dental and surgical procedures. The valvular lesions can progress, and regular follow-up cardiovascular evaluation should be planned. Finally, despite potential problems with tissue friability and healing and a possible tendency for increased bleeding, successful valve replacement can be carried out if necessitated by cardiac disability.

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There are more references available in the full text version of this article.

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  • Heart disease in patients with osteogenesis imperfecta - A systematic review

    2015, International Journal of Cardiology
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    One additional study was found through the reference lists of the included articles. Thus, a total of 68 studies were included in this review, comprising 51 case reports of OI [15–64], 8 small OI case series (containing less than 10 patients) [65–72], 4 large OI case series (containing 10 or more patients) [13,73–75] and 5 cross-sectional comparative studies including controls [76–80]. In the case reports and case series and all but one cross-sectional study, the OI diagnosis was based on the patient's clinical characteristics.

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    Considering the more pronounced enlargement of RV and PA1 dimensions in the present study and enlargement of the left ventricle and aorta as previously demonstrated,6 we suggest regular follow-ups by cardiologists, especially in these patients. Increased frequency of mitral and aortic regurgitations in OI has previously been demonstrated.5,6,25 Although 2 patients with OI had moderate TR and no controls had TR, we could not show the same increased regurgitations for the tricuspid and pulmonic valves as on the left side.6

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Supported in part by Program Project Grant HL 06336 and Medical Cardiology Training Grant HL 05791 of the United States Public Health Service.

1

From the Division of Cardiology, Department of Medicine, University of Oregon Health Sciences Center, Portland, Ore.

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