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A Case of Cardiomyopathy Based on Limb-girdle Muscular Dystrophy in which Mitral Valvoplasty Together with Left Ventriculectomy was Relieved from Catecholamine-dependent Heart Failure Akira Sato 1 , Takayuki Inomata 1 , Toshimi Koitabashi 1 , Hisahito Shinagawa 1 , Nishii Mototsugu 1 , Naoyoshi Aoyama 1 , Tadashi Isomura 2 , Tohru Izumi 1 1Department of Cardio-Angiology, Kitasato University School of Medicine 2Department of Cardiovascular Surgery, Hayama Heart Center Keyword: 筋ジストロフィー心筋症 , 僧帽弁閉鎖不全 , 左室形成術 , cardiomyopathy based on muscular dystrophy , mitral regurgitation , left ventriculectomy pp.843-847
Published Date 2010/8/15
DOI https://doi.org/10.11477/mf.1404101533
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 A fifty-three-year-old woman with dilated cardiomyopathy caused by limb-girdle type muscular dystrophy suffered from refractory congestive heart failure(HF)and was treated with an intravenous catecholamine infusion. Assessing her long-term course indicated by echocardiographic and clinical parameters, an aneurysmal formation of the posterior left ventricular(LV)wall leading to advanced mitral valve(MV)regurgitation caused by MV tethering in addition to impaired LV contraction was found and factors of HF exacerbation. Based on the above understanding, partial left ventriculectomy using posterior wall excision and mitral annuloplasty were designated. The operation succeeded in the catecholamine secession together with intensified medical treatment for HF.

 This is a representative case of muscular dystrophy/cardiomyopathy in which ventriculectomy of the aneurysmal portion and mitral annuloplasty was able to reduce MV regurgitation derived from MV tethering, leading to relief of exacerbated HF.


Copyright © 2010, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1200 印刷版ISSN 0452-3458 医学書院

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