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针对伴有层粘连蛋白相关肌肉萎缩症的晚期心力衰竭患者的吸气肌训练

Inspiratory muscle training for advanced heart failure with lamin-related muscular dystrophy.

作者信息

Taya Masanobu, Amiya Eisuke, Hatano Masaru, Maki Hisataka, Hosoya Yumiko, Ishida Junichi, Bujo Chie, Tsuji Masaki, Konishi Yuto, Yokota Kazuhiko, Haga Nobuhiko, Komuro Issei

机构信息

Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

Department of Rehabilitation Medicine, The University of Tokyo Hospital, Tokyo, Japan.

出版信息

J Cardiol Cases. 2019 Oct 29;20(6):232-234. doi: 10.1016/j.jccase.2019.09.007. eCollection 2019 Dec.

DOI:10.1016/j.jccase.2019.09.007
PMID:31762841
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6859540/
Abstract

Respiratory muscle weakness is often complicated in patients with heart failure. Its presence further worsens the clinical course of heart failure. However, the effect and appropriate method of inspiratory muscle training has not previously been elucidated. A 55-year-old man with dilated cardiomyopathy was admitted for intractable heart failure. His heart failure was dependent on catecholamine infusion and the implantation of left ventricular assist device was planned. He also had suffered from some muscle weakness, which was later diagnosed as lamin dystrophy due to mutation of c.G97T E33X. Preoperatively we started aerobic rehabilitation with inspiratory muscle training. Before training, inspiratory and expiratory muscle strength was significantly reduced and exercise capacity was decreased. The load of inspiratory training could be gradually increased along the result of regular evaluation of respiratory muscle strength. During 8 weeks of training, there was no worsening of heart failure and no significant events related to arrhythmia. After training, respiratory muscle strength and exercise capacity were improved significantly. < Inspiratory muscle training was effective and safe in a patient with intractable heart failure, which was complicated by skeletal muscle myopathy due to lamin-related muscular dystrophy.>.

摘要

呼吸肌无力在心力衰竭患者中常并发。其存在会进一步恶化心力衰竭的临床病程。然而,吸气肌训练的效果和合适方法此前尚未阐明。一名55岁扩张型心肌病男性因顽固性心力衰竭入院。他的心力衰竭依赖儿茶酚胺输注,计划植入左心室辅助装置。他还患有一些肌肉无力,后来因c.G97T E33X突变被诊断为lamin营养不良。术前我们开始进行有氧康复并进行吸气肌训练。训练前,吸气和呼气肌力量显著降低,运动能力下降。根据呼吸肌力量的定期评估结果,吸气训练的负荷可逐渐增加。在8周的训练期间,心力衰竭没有恶化,也没有与心律失常相关的重大事件。训练后,呼吸肌力量和运动能力显著提高。<吸气肌训练对一名因lamin相关肌肉营养不良并发骨骼肌肌病的顽固性心力衰竭患者有效且安全。>

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