Article
Refractory Right Ventricular Failure in a Patient with Emery-Dreifuss Muscular Dystrophy.
Internal medicine (Tokyo, Japan) - 15 May 2020
Chiba Yasuyuki, Fukushima Arata, Nakao Motoki, Kobayashi Yuta, Ishigaki Takahiro, Tenma Taro, Kamiya Kiwamu, Shingu Yasushige, Ooka Tomonori, Matsui Yoshiro, Anzai Toshihisa
Abstract excerpt
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...
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