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Cardiac sarcoidosis mimics cardiac amyloidosis in an elderly patient

Cardiac sarcoidosis mimics cardiac amyloidosis in an elderly patient A 70-year-old man with long-standing heart failure with preserved ejection fraction (HFpEF), atrial fibrillation, aortic stenosis, and stage III chronic kidney disease was admitted after presenting with syncope and a heart failure exacerbation. He had recently had cardiac magnetic resonance imaging (CMR) that raised the possibility of cardiac amyloidosis. This was based on late gadolinium enhancement (LGE) in a diffuse pattern, along with concentric increased wall thickness (Figure 1A). There was no definitively increased left ventricular myocardial signal on T2 oedema weighted imaging, although, the presence of increased extracellular volume was supported by T1 mapping, which showed slightly elevated native T1 values of 1171 ± 296 ms (Figure 1B). However, Tc-99m-pyrophosphate was negative for TTR amyloidosis. Serum and urine electrophoresis, serum-free light chains, and immunofixation were also negative for AL amyloidosis. He next underwent cardiac FDG-positron emission tomography (FDG-PET) that revealed inflamed myocardium in the basal inferoseptum, basal inferior segments, basal interatrial septum, and left atrium (Figure 1C). There were also FDG avid mediastinal lymph nodes. This raised the suspicion of cardiac sarcoidosis as a unifying diagnosis. Endobronchial biopsy of lymph nodes revealed non-caseating granulomas, consistent with sarcoidosis (Figure 1D). This patient’s clinical presentation of advanced age, HFpEF, atrial fibrillation, aortic stenosis, renal failure, and diffuse LGE on CMR initially raised the possibility of cardiac amyloidosis. However, FDG-PET and biopsy reclassified the diagnosis to cardiac sarcoidosis. This case demonstrates that sarcoidosis is a great mimicker and may present with cardiac LGE patterns similar to cardiac amyloidosis. Figure 1 Open in new tabDownload slide (A) CMR revealed concentric increased left ventricular wall thickness and diffuse late gadolinium enhancement in the basal to mid-myocardial segments with relative sparing of the apex, suggestive of cardiac amyloidosis rather than a cardiac sarcoidosis pattern. (B) CMR native T1 mapping values were slightly elevated at 1171 ± 296 ms. (C) FDG-PET showed a large area of increased signal in the basal inferoseptum, basal inferior segments, lateral wall, basal interatrial septum, posterior left atrial wall, and left atrial appendage. (D) Mediastinal lymph node biopsy via endobronchial ultrasound guidance identified non-caseating granulomas consistent with sarcoidosis. Cohesive aggregates of epithelioid histiocytes in a polymorphous lymphoid background are seen (Romanowsky stain, 100x magnification). Supplementary material Supplementary material is available at European Heart Journal – Case Reports. Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with COPE guidance. Funding: None declared Data availability The data underlying this article are available in the article and in its online Supplementary material. Author notes Conflict of interest: None declared. © The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal - Case Reports Oxford University Press

Cardiac sarcoidosis mimics cardiac amyloidosis in an elderly patient

Cardiac sarcoidosis mimics cardiac amyloidosis in an elderly patient

European Heart Journal - Case Reports , Volume 6 (12): 1 – Dec 6, 2022

Abstract

A 70-year-old man with long-standing heart failure with preserved ejection fraction (HFpEF), atrial fibrillation, aortic stenosis, and stage III chronic kidney disease was admitted after presenting with syncope and a heart failure exacerbation. He had recently had cardiac magnetic resonance imaging (CMR) that raised the possibility of cardiac amyloidosis. This was based on late gadolinium enhancement (LGE) in a diffuse pattern, along with concentric increased wall thickness (Figure 1A). There was no definitively increased left ventricular myocardial signal on T2 oedema weighted imaging, although, the presence of increased extracellular volume was supported by T1 mapping, which showed slightly elevated native T1 values of 1171 ± 296 ms (Figure 1B). However, Tc-99m-pyrophosphate was negative for TTR amyloidosis. Serum and urine electrophoresis, serum-free light chains, and immunofixation were also negative for AL amyloidosis. He next underwent cardiac FDG-positron emission tomography (FDG-PET) that revealed inflamed myocardium in the basal inferoseptum, basal inferior segments, basal interatrial septum, and left atrium (Figure 1C). There were also FDG avid mediastinal lymph nodes. This raised the suspicion of cardiac sarcoidosis as a unifying diagnosis. Endobronchial biopsy of lymph nodes revealed non-caseating granulomas, consistent with sarcoidosis (Figure 1D). This patient’s clinical presentation of advanced age, HFpEF, atrial fibrillation, aortic stenosis, renal failure, and diffuse LGE on CMR initially raised the possibility of cardiac amyloidosis. However, FDG-PET and biopsy reclassified the diagnosis to cardiac sarcoidosis. This case demonstrates that sarcoidosis is a great mimicker and may present with cardiac LGE patterns similar to cardiac amyloidosis. Figure 1 Open in new tabDownload slide (A) CMR revealed concentric increased left ventricular wall thickness and diffuse late gadolinium enhancement in the basal to mid-myocardial segments with relative sparing of the apex, suggestive of cardiac amyloidosis rather than a cardiac sarcoidosis pattern. (B) CMR native T1 mapping values were slightly elevated at 1171 ± 296 ms. (C) FDG-PET showed a large area of increased signal in the basal inferoseptum, basal inferior segments, lateral wall, basal interatrial septum, posterior left atrial wall, and left atrial appendage. (D) Mediastinal lymph node biopsy via endobronchial ultrasound guidance identified non-caseating granulomas consistent with sarcoidosis. Cohesive aggregates of epithelioid histiocytes in a polymorphous lymphoid background are seen (Romanowsky stain, 100x magnification). Supplementary material Supplementary material is available at European Heart Journal – Case Reports. Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with COPE guidance. Funding: None declared Data availability The data underlying this article are available in the article and in its online Supplementary material. Author notes Conflict of interest: None declared. © The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

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Publisher
Oxford University Press
Copyright
Copyright © 2023 European Society of Cardiology
eISSN
2514-2119
DOI
10.1093/ehjcr/ytac458
Publisher site
See Article on Publisher Site

Abstract

A 70-year-old man with long-standing heart failure with preserved ejection fraction (HFpEF), atrial fibrillation, aortic stenosis, and stage III chronic kidney disease was admitted after presenting with syncope and a heart failure exacerbation. He had recently had cardiac magnetic resonance imaging (CMR) that raised the possibility of cardiac amyloidosis. This was based on late gadolinium enhancement (LGE) in a diffuse pattern, along with concentric increased wall thickness (Figure 1A). There was no definitively increased left ventricular myocardial signal on T2 oedema weighted imaging, although, the presence of increased extracellular volume was supported by T1 mapping, which showed slightly elevated native T1 values of 1171 ± 296 ms (Figure 1B). However, Tc-99m-pyrophosphate was negative for TTR amyloidosis. Serum and urine electrophoresis, serum-free light chains, and immunofixation were also negative for AL amyloidosis. He next underwent cardiac FDG-positron emission tomography (FDG-PET) that revealed inflamed myocardium in the basal inferoseptum, basal inferior segments, basal interatrial septum, and left atrium (Figure 1C). There were also FDG avid mediastinal lymph nodes. This raised the suspicion of cardiac sarcoidosis as a unifying diagnosis. Endobronchial biopsy of lymph nodes revealed non-caseating granulomas, consistent with sarcoidosis (Figure 1D). This patient’s clinical presentation of advanced age, HFpEF, atrial fibrillation, aortic stenosis, renal failure, and diffuse LGE on CMR initially raised the possibility of cardiac amyloidosis. However, FDG-PET and biopsy reclassified the diagnosis to cardiac sarcoidosis. This case demonstrates that sarcoidosis is a great mimicker and may present with cardiac LGE patterns similar to cardiac amyloidosis. Figure 1 Open in new tabDownload slide (A) CMR revealed concentric increased left ventricular wall thickness and diffuse late gadolinium enhancement in the basal to mid-myocardial segments with relative sparing of the apex, suggestive of cardiac amyloidosis rather than a cardiac sarcoidosis pattern. (B) CMR native T1 mapping values were slightly elevated at 1171 ± 296 ms. (C) FDG-PET showed a large area of increased signal in the basal inferoseptum, basal inferior segments, lateral wall, basal interatrial septum, posterior left atrial wall, and left atrial appendage. (D) Mediastinal lymph node biopsy via endobronchial ultrasound guidance identified non-caseating granulomas consistent with sarcoidosis. Cohesive aggregates of epithelioid histiocytes in a polymorphous lymphoid background are seen (Romanowsky stain, 100x magnification). Supplementary material Supplementary material is available at European Heart Journal – Case Reports. Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with COPE guidance. Funding: None declared Data availability The data underlying this article are available in the article and in its online Supplementary material. Author notes Conflict of interest: None declared. © The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

Journal

European Heart Journal - Case ReportsOxford University Press

Published: Dec 6, 2022

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