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Published on: May 16, 2020
Desmin-related restrictive cardiomyopathy.
Christiano Pereira Silva1, Fernando Bacal, Luiz Alberto Benvenuti
1Instituto do Coração, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil. chrispsilva@cardiol.br
Arquivos Brasileiros De Cardiologia
|March 5, 2008
Summary
Restrictive cardiomyopathies can stem from desmin accumulation, a rare condition. This case highlights desmin storage disease presenting initially with heart block, progressing to muscle issues and heart failure.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Restrictive cardiomyopathies encompass various etiologies, including storage diseases.
- Desmin accumulation (desminopathies) is an uncommon cause of cardiac and muscle dysfunction.
Observation:
- This report details a patient whose initial symptom was complete atrioventricular block.
- The patient later exhibited skeletal muscle alterations and developed heart failure.
Findings:
- Diagnostic investigation confirmed restrictive cardiomyopathy.
- The underlying cause was identified as desmin accumulation.
Implications:
- This case underscores the importance of considering desmin storage disease in patients with unexplained restrictive cardiomyopathy and conduction abnormalities.
- Early diagnosis of desminopathies is crucial for managing cardiac and peripheral myopathies.
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