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Isolated cardiac amyloidosis, or "Stiff Heart Syndrome," is a rare manifestation of amyloidosis. Some degree of cardiac amyloid deposition is common in elderly patients, as reported in prior post-mortem studies; however, isolated cardiac involvement with predominantly cardiac symptoms and no evidence of systemic disease is a rare presentation. Establishing the correct diagnosis, even with the use of extensive testing including amyloid typing, understanding the clinical significance, and management can be challenging in such cases.


Satya S Bhupathi, Sreelatha Chalasani, Roxann Rokey. Stiff heart syndrome. Clinical medicine & research. 2011 Jun;9(2):92-9

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PMID: 20852084

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