• Yasuyuki Chiba, Arata Fukushima, Motoki Nakao, Yuta Kobayashi, Takahiro Ishigaki, Taro Tenma, Kiwamu Kamiya, Yasushige Shingu, Tomonori Ooka, Yoshiro Matsui, and Toshihisa Anzai.
• Department of Cardiovascular Medicine, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.
• Intern. Med. 2020 May 15; 59 (10): 1277-1281.

AbstractA 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.

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