Tafamidis for the Treatment of Hereditary Transthyretin Amyloid Cardiomyopathy: A Case Report

被引:6
|
作者
Fujita, Teppei [1 ]
Inomata, Takayuki [3 ]
Kaida, Toyoji [1 ]
Iida, Yuichirou [1 ]
Ikeda, Yuki [1 ]
Nabeta, Takeru [1 ]
Ishii, Shunsuke [1 ]
Maekawa, Emi [1 ]
Naruke, Takashi [1 ]
Koitabashi, Toshimi [1 ]
Kitamura, Eiji [2 ]
Sekijima, Yoshiki [4 ]
Ako, Junya [1 ]
机构
[1] Kitasato Univ, Sch Med, Dept Cardiovasc Med, Sagamihara, Kanagawa, Japan
[2] Kitasato Univ, Sch Med, Dept Neurol, Sagamihara, Kanagawa, Japan
[3] Kitasato Univ, Kitasato Inst Hosp, Dept Cardiovasc Med, Tokyo, Japan
[4] Shinshu Univ, Dept Med Neurol & Rheumatol, Sch Med, Matsumoto, Nagano, Japan
关键词
Amyloidosis; Hereditary transthyretin; Tafamidis; Amyloid cardiomyopathy; POLYNEUROPATHY; NON-VAL30MET; TRIAL; AL;
D O I
10.1159/000455089
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Tafamidis meglumine is a novel medicine that has been shown to slow the progression of peripheral neurological impairment in patients with hereditary transthyretin amyloidosis (ATTR). However, the efficacy of tafamidis against ATTR-related cardiac amyloidosis remains unclear. A 72-year-old woman had cardiac hypertrophy and axonopathy in her lower legs. Endomyocardial biopsy revealed an infiltrative cardiomyopathy consistent with amyloidosis. Immunostaining and genetic studies confirmed the diagnosis of ATTR, and tafamidis was started subsequently. Two years after the initiation of tafamidis treatment, electromyography demonstrated no change in the axonopathy in her lower legs; however, electrocardiography displayed QRS prolongation, and echocardiography disclosed an increase in interventricular septal thickness. Endomyocardial biopsy indicated that transthyretin amyloid infiltration of the myocardium was not reduced. In this case, there was no apparent progression of axonopathy, although there were signs of worsening amyloid cardiomyopathy during the treatment with tafamidis. (C) 2017 S. Karger AG, Basel
引用
收藏
页码:74 / 77
页数:4
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