Myocardial Involvement in Patients With Osteogenesis Imperfecta

Katsura Soma; Hajime Abe; Norihiko Takeda; Yukako Shintani; Yutaka Takazawa; Toshiya Kojima; Katsuhito Fujiu; Hiroaki Semba; Hiroshi Yamashita; Yasunobu Hirata; Masashi Fukayama; Ryozo Nagai

doi:10.1536/ihj.53.75

Case Reports

Myocardial Involvement in Patients With Osteogenesis Imperfecta

Katsura Soma, Hajime Abe, Norihiko Takeda, Yukako Shintani, Yutaka Takazawa, Toshiya Kojima, Katsuhito Fujiu, Hiroaki Semba, Hiroshi Yamashita, Yasunobu Hirata, Masashi Fukayama, Ryozo Nagai

Author information

Katsura Soma
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Hajime Abe
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Norihiko Takeda
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Yukako Shintani
Department of Pathology, Faculty of Medicine, The University of Tokyo
Yutaka Takazawa
Department of Pathology, Faculty of Medicine, The University of Tokyo
Toshiya Kojima
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Katsuhito Fujiu
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Hiroaki Semba
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Hiroshi Yamashita
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Yasunobu Hirata
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
Masashi Fukayama
Department of Pathology, Faculty of Medicine, The University of Tokyo
Ryozo Nagai
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo

Keywords: Osteogenesis imperfecta, Type 1 collagen, Mitral valve regurgitation, Myocardial fibrosis

JOURNAL FREE ACCESS

2012 Volume 53 Issue 1 Pages 75-77

DOI https://doi.org/10.1536/ihj.53.75

Details

Abstract

Mitral and aortic valve regurgitation is commonly found in osteogenesis imperfecta (OI) patients, however, little is known about the myocardial involvement in this disorder. An 82-year-old man with OI developed heart failure and was admitted to our hospital. Echocardiogram revealed severe mitral regurgitation without left ventricular (LV) dilatation, but with LV wall thickening. Histological analysis exhibited interstitial fibrosis of the myocardium in addition to myxoid changes of the mitral leaflet. These findings suggest that OI patients may develop LV remodeling together with diastolic dysfunction.

Corresponding author

Register with J-STAGE for free!