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Noteworthy Cardiovascular Involvement with Sporadic Late-onset Nemaline Myopathy.
Kosuke Matsuzono1, Theerawat Kumutpongpanich2, Kana Kubota3
1Division of Neurology, Department of Medicine, Jichi Medical University School of Medicine, Japan.
Sporadic late-onset nemaline myopathy (SLONM) can cause severe cardiac issues, including cardiac arrest and pulmonary hypertension. This case highlights the critical need to consider cardiovascular complications in SLONM diagnosis.
Area of Science:
- Cardiology
- Neurology
- Rare Diseases
Background:
- Sporadic late-onset nemaline myopathy (SLONM) is a rare neuromuscular disorder.
- Cardiac involvement is an emerging concern in SLONM.
- Understanding SLONM's cardiovascular manifestations is crucial for patient management.
Observation:
- A 66-year-old woman presented with recurrent cardiac arrest, right ventricular failure, and pulmonary hypertension.
- Despite pacemaker implantation and positive airway pressure, her cardiac symptoms persisted.
- Diagnosis of SLONM was confirmed via muscle biopsy.
Findings:
- The patient's primary symptoms were indicative of significant cardiovascular compromise.
- SLONM was the underlying cause of the severe cardiac manifestations.
- Right ventricular failure and pulmonary hypertension were prominent features.
Implications:
- This case underscores the potential for severe cardiac involvement in SLONM.
- It emphasizes the importance of cardiac evaluation in patients with unexplained heart failure and arrhythmias.
- Early recognition and management of cardiovascular complications may improve outcomes for SLONM patients.
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