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Refractory Right Ventricular Failure in a Patient with Emery-Dreifuss Muscular Dystrophy

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Title: Refractory Right Ventricular Failure in a Patient with Emery-Dreifuss Muscular Dystrophy
Authors: Chiba, Yasuyuki Browse this author
Fukushima, Arata Browse this author →KAKEN DB
Nakao, Motoki Browse this author
Kobayashi, Yuta Browse this author
Ishigaki, Takahiro Browse this author
Tenma, Taro Browse this author →KAKEN DB
Kamiya, Kiwamu Browse this author →KAKEN DB
Shingu, Yasushige Browse this author →KAKEN DB
Ooka, Tomonori Browse this author →KAKEN DB
Matsui, Yoshiro Browse this author →KAKEN DB
Anzai, Toshihisa Browse this author →KAKEN DB
Keywords: right ventricular failure
Emery-Dreifuss muscular dystrophy
left ventricular assist device
heart transplantation
Issue Date: 15-May-2020
Publisher: 日本内科学会
Journal Title: Internal medicine
Volume: 59
Issue: 10
Start Page: 1277
End Page: 1281
Publisher DOI: 10.2169/internalmedicine.4113-19
Abstract: A 23-year-old man had progressive muscle weakness and Emery-Dreifuss muscular dystrophy (EDMD) due to a LMNA (lamin A/C) mutation. Congestive heart failure diagnosed at 19 years of age. Maximal drug treatment/cardiac resynchronization failed to improve the cardiac function. He was therefore hospitalized due to heart failure. Despite extracorporeal membrane oxygenation, he developed severe right heart dysfunction and died (multiple organ failure). A cardiac lesion's presence determines the prognosis of EDMD. While there are many arrhythmia reports, few reports on heart failure (particularly severe heart failure requiring cardiac transplantation) have been published. Right heart function monitoring and early ventricular-assist device use plus right heart support considering heart transplantation are important.
Rights: by-nc-nd/4.0/
Type: article
Appears in Collections:医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

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