Key result
Sporadic late-onset nemaline myopathy can present with significant cardiovascular involvement, including right ventricular failure, pulmonary hypertension, and repetitive cardiac arrest.
Why the study?
Cardiac involvement has recently been the focus of sporadic late-onset nemaline myopathy (SLONM), with right ventricular failure, pulmonary hypertension, and repetitive cardiac arrest being noteworthy characteristics.
Case Report (n=1)
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Sporadic late-onset nemaline myopathy should be considered in patients presenting with unexplained right ventricular failure, pulmonary hypertension, and repetitive cardiac arrest.
Cardiac 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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Matsuzono et al. (2021) conducted a case report in Sporadic late-onset nemaline myopathy (SLONM) (n=1). Sporadic late-onset nemaline myopathy (SLONM) was evaluated. Sporadic late-onset nemaline myopathy can present with significant cardiovascular involvement, including right ventricular failure, pulmonary hypertension, and repetitive cardiac arrest.
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