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Hokkaido University Collection of Scholarly and Academic Papers >
Graduate School of Medicine / Faculty of Medicine >
Peer-reviewed Journal Articles, etc >
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: | https://creativecommons.org/licenses/ by-nc-nd/4.0/ |
| Type: | article |
| URI: | http://hdl.handle.net/2115/78879 |
| 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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