Potential causes of sudden cardiac death in nemaline myopathy

Finsterer, Josef; Frank, Marlies

doi:10.1186/s13052-015-0175-x

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Myopathies' Effect On the Cardiopulmonary Interaction1

Nemaline Myopathy: From Clinical Suspicion To Cardiopulmonar...1

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2022

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Cited by 1 publication

(2 citation statements)

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“…Some myopathy-specific medications may have beneficial effects on cardiac involvement. For example, steroids can be used in DMD patients and may be effective in delaying the onset of cardiomyopathy ( 6 , 53 , 54 ). Enzyme replacement therapy (ERT) can be beneficial in Pompe's disease complicated with hypertrophic cardiomyopathy ( 55 ).…”

Section: Myopathies' Effect On the Cardiopulmonary Interactionmentioning

confidence: 99%

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Disruption of cardio-pulmonary coupling in myopathies: Pathophysiological and mechanistic characterization with special emphasis on nemaline myopathy

Ronderos-Botero

Dileep

Yapor

et al. 2022

Front. Cardiovasc. Med.

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The heart and lung are in continuous reciprocal interaction that creates a functional and anatomical reserve referred to as cardiopulmonary coupling (CPC). Disruption of CPC can occur due to various cardiac or pulmonary pathologies but also can occur in patients with myopathies. Nemaline myopathy (NM) is a skeletal muscle heterogeneous disorder due to contractile proteins' gene mutations that impact lung and cardiac mechanics and thus is expected to adversely affect CPC in a complex manner. We present a case of NM and we review the literature on cardiac and pulmonary effects of myopathy-related disruption of CPC.

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Section: Myopathies' Effect On the Cardiopulmonary Interactionmentioning

confidence: 99%

“…In a recent review of literature for causes of sudden cardiac death in Nemaline myopathy ( 53 ), types and frequencies of cardiac involvement were identified in 35 patients. Of these patients, 22 were male and 8 were female.…”

Section: Nemaline Myopathy: From Clinical Suspicion To Cardiopulmonar...mentioning

confidence: 99%