Cardiac AA amyloidosis in a patient with obstructive hypertrophic cardiomyopathy
Copyright © 2020 Elsevier Inc. All rights reserved..
Cardiac amyloid A (AA) amyloidosis is rare. We present the case of a 72-year-old woman with obstructive hypertrophic cardiomyopathy (HCM) and biopsy-proven renal AA amyloidosis whose dyspnea and exercise intolerance had worsened over the previous year. Her AA amyloidosis was suspected to be secondary to chronic diverticulitis for which she had undergone hemicolectomy and sigmoidectomy 3 years prior. Echocardiographic findings were consistent with worsening left ventricular outflow tract obstruction at rest. Cardiac magnetic resonance imaging revealed patchy areas of midwall late gadolinium enhancement. Right ventricular endomyocardial biopsy did not reveal amyloid deposition, and cardiac technetium-99m pyrophosphate scintigraphy did not suggest transthyretin amyloidosis. The patient underwent septal myectomy with resection of an accessory papillary muscle. Pathological examination of the myectomy specimen was consistent with HCM. In addition, there was a thick layer of diffuse endocardial and vascular amyloid deposition that was identified as AA type by laser-microdissection with liquid chromatography-coupled tandem-mass spectrometry. This case report highlights the presence of 2 distinct disease processes occurring simultaneously and the importance of tissue diagnosis of AA amyloidosis, a condition that is not commonly associated with HCM.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:48 |
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| Enthalten in: |
Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology - 48(2020) vom: 05. Sept., Seite 107218 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Li, Boyangzi [VerfasserIn] |
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| Links: |
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| Themen: |
AA amyloidosis |
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| Anmerkungen: |
Date Completed 01.09.2020 Date Revised 01.09.2020 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.carpath.2020.107218 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM309716616 |
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| 520 | |a Copyright © 2020 Elsevier Inc. All rights reserved. | ||
| 520 | |a Cardiac amyloid A (AA) amyloidosis is rare. We present the case of a 72-year-old woman with obstructive hypertrophic cardiomyopathy (HCM) and biopsy-proven renal AA amyloidosis whose dyspnea and exercise intolerance had worsened over the previous year. Her AA amyloidosis was suspected to be secondary to chronic diverticulitis for which she had undergone hemicolectomy and sigmoidectomy 3 years prior. Echocardiographic findings were consistent with worsening left ventricular outflow tract obstruction at rest. Cardiac magnetic resonance imaging revealed patchy areas of midwall late gadolinium enhancement. Right ventricular endomyocardial biopsy did not reveal amyloid deposition, and cardiac technetium-99m pyrophosphate scintigraphy did not suggest transthyretin amyloidosis. The patient underwent septal myectomy with resection of an accessory papillary muscle. Pathological examination of the myectomy specimen was consistent with HCM. In addition, there was a thick layer of diffuse endocardial and vascular amyloid deposition that was identified as AA type by laser-microdissection with liquid chromatography-coupled tandem-mass spectrometry. This case report highlights the presence of 2 distinct disease processes occurring simultaneously and the importance of tissue diagnosis of AA amyloidosis, a condition that is not commonly associated with HCM | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a AA amyloidosis | |
| 650 | 4 | |a Cardiac amyloidosis | |
| 650 | 4 | |a Hypertrophic cardiomyopathy | |
| 650 | 4 | |a Septal myectomy | |
| 650 | 7 | |a Serum Amyloid A Protein |2 NLM | |
| 700 | 1 | |a Ahluwalia, Monica |e verfasserin |4 aut | |
| 700 | 1 | |a Narula, Navneet |e verfasserin |4 aut | |
| 700 | 1 | |a Moreira, Andre L |e verfasserin |4 aut | |
| 700 | 1 | |a Swistel, Daniel G |e verfasserin |4 aut | |
| 700 | 1 | |a Massera, Daniele |e verfasserin |4 aut | |
| 700 | 1 | |a Sherrid, Mark V |e verfasserin |4 aut | |
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