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Case Reports
. 2011 Jun;9(2):92-9.
doi: 10.3121/cmr.2010.899. Epub 2010 Sep 17.

Stiff heart syndrome

Satya S Bhupathi  1 , Sreelatha Chalasani, Roxann Rokey
Affiliations
Case Reports

Stiff heart syndrome

Satya S Bhupathi et al. Clin Med Res. 2011 Jun.

Abstract

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.

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Figures

Figure 1.
Figure 1.
Endomyocardial biopsy showing extensive amyloid deposition as pale eosinophilic material surrounding myocardial cells (H&E, x40).
Figure 2.
Figure 2.
Endomyocardial biopsy showing extensive amyloid deposition as pale eosinophilic material surrounding distorted myocardial cells (H&E, x40).
Figure 3.
Figure 3.
Orange-pink staining amyloid which exhibited yellow-green birefringence under polarized light (not shown) (Congo red, x40).
See this image and copyright information in PMC

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