• Kosuke Matsuzono, Theerawat Kumutpongpanich, Kana Kubota, Takafumi Okuyama, Kohei Furuya, Tomoya Yagisawa, Akie Horikiri, Takeshi Igarashi, Kumiko Miura, Tadashi Ozawa, Takafumi Mashiko, Haruo Shimazaki, Reiji Koide, Ryota Tanaka, Hayato Shimizu, Yasushi Imai, Kazuomi Kario, Ichizo Nishino, and Shigeru Fujimoto.
• Division of Neurology, Department of Medicine, Jichi Medical University School of Medicine, Japan.
• Intern. Med. 2021 Jul 15; 60 (14): 2327-2332.

AbstractCardiac involvement has recently been the focus of sporadic late-onset nemaline myopathy (SLONM). However, right ventricular failure and pulmonary hypertension, in addition to repetitive cardiac arrest, are noteworthy characteristics of SLONM. We herein report a 66-year-old woman with SLONM whose main symptoms were cardiac arrest, right ventricular failure, and pulmonary hypertension. Despite permanent pacemaker replacement, cardiac arrest occurred repetitively, and even with continuous positive airway pressure, right ventricular failure and pulmonary hypertension persisted. The patient was finally diagnosed with SLONM by a muscle biopsy. Our case suggests the possibility of cardiovascular involvement in SLONM, especially right ventricular failure and pulmonary hypertension.

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