Multimodal Imaging and Biomarkers in Cardiac Amyloidosis
Amyloidosis is a progressive infiltrative disease instigated by the extracellular deposition of amyloid fibrils in various organs such as the heart, kidney, and peripheral nerves. Cardiac amyloid deposits cause restrictive cardiomyopathy, leading to a poor prognosis in systemic amyloidosis. The most...
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MDPI AG
2022-03-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/12/3/627 |
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| author | Mi-Hyang Jung Suyon Chang Eun Ji Han Jong-Chan Youn |
| author_facet | Mi-Hyang Jung Suyon Chang Eun Ji Han Jong-Chan Youn |
| author_sort | Mi-Hyang Jung |
| collection | DOAJ |
| description | Amyloidosis is a progressive infiltrative disease instigated by the extracellular deposition of amyloid fibrils in various organs such as the heart, kidney, and peripheral nerves. Cardiac amyloid deposits cause restrictive cardiomyopathy, leading to a poor prognosis in systemic amyloidosis. The most common etiologies of cardiac amyloidosis (CA) are immunoglobulin light chain deposits (AL-CA) and misfolded transthyretin deposits (ATTR-CA). In recent years, many developments have been accomplished in the field of diagnosis and treatment of CA. At present, ATTR-CA can be noninvasively diagnosed if the following two conditions are fulfilled in the setting of typical echocardiographic/cardiac MRI findings: (1) grade 2 or 3 myocardial uptake in bone scintigraphy confirmed by SPECT and (2) absence of monoclonal protein confirmed by serum-free light chain assay, and serum/urine protein electrophoresis with immunofixation test. Effective therapies are evolving in both types of CA (tafamidis for ATTR-CA and immunologic treatments for AL-CA). Thus, early suspicion and prompt diagnosis are crucial for achieving better outcomes. In this review, we have summarized the role of multimodal imaging (e.g., echocardiography, cardiac MRI, and bone scintigraphy) and biomarkers (e.g., troponin, BNP) in the diagnosis, risk stratification, and treatment monitoring of CA. |
| first_indexed | 2024-03-09T19:57:31Z |
| format | Article |
| id | doaj.art-f1d4275745f04a958b5bef2c60dd98a9 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-09T19:57:31Z |
| publishDate | 2022-03-01 |
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| spelling | doaj.art-f1d4275745f04a958b5bef2c60dd98a92023-11-24T00:54:59ZengMDPI AGDiagnostics2075-44182022-03-0112362710.3390/diagnostics12030627Multimodal Imaging and Biomarkers in Cardiac AmyloidosisMi-Hyang Jung0Suyon Chang1Eun Ji Han2Jong-Chan Youn3Division of Cardiology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, KoreaDepartment of Radiology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, KoreaDivision of Nuclear Medicine, Department of Radiology, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, KoreaDivision of Cardiology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, KoreaAmyloidosis is a progressive infiltrative disease instigated by the extracellular deposition of amyloid fibrils in various organs such as the heart, kidney, and peripheral nerves. Cardiac amyloid deposits cause restrictive cardiomyopathy, leading to a poor prognosis in systemic amyloidosis. The most common etiologies of cardiac amyloidosis (CA) are immunoglobulin light chain deposits (AL-CA) and misfolded transthyretin deposits (ATTR-CA). In recent years, many developments have been accomplished in the field of diagnosis and treatment of CA. At present, ATTR-CA can be noninvasively diagnosed if the following two conditions are fulfilled in the setting of typical echocardiographic/cardiac MRI findings: (1) grade 2 or 3 myocardial uptake in bone scintigraphy confirmed by SPECT and (2) absence of monoclonal protein confirmed by serum-free light chain assay, and serum/urine protein electrophoresis with immunofixation test. Effective therapies are evolving in both types of CA (tafamidis for ATTR-CA and immunologic treatments for AL-CA). Thus, early suspicion and prompt diagnosis are crucial for achieving better outcomes. In this review, we have summarized the role of multimodal imaging (e.g., echocardiography, cardiac MRI, and bone scintigraphy) and biomarkers (e.g., troponin, BNP) in the diagnosis, risk stratification, and treatment monitoring of CA.https://www.mdpi.com/2075-4418/12/3/627amyloidosisimmunoglobulin light chain amyloidosistransthyretin amyloidosismultimodal imagingbiomarkers |
| spellingShingle | Mi-Hyang Jung Suyon Chang Eun Ji Han Jong-Chan Youn Multimodal Imaging and Biomarkers in Cardiac Amyloidosis Diagnostics amyloidosis immunoglobulin light chain amyloidosis transthyretin amyloidosis multimodal imaging biomarkers |
| title | Multimodal Imaging and Biomarkers in Cardiac Amyloidosis |
| title_full | Multimodal Imaging and Biomarkers in Cardiac Amyloidosis |
| title_fullStr | Multimodal Imaging and Biomarkers in Cardiac Amyloidosis |
| title_full_unstemmed | Multimodal Imaging and Biomarkers in Cardiac Amyloidosis |
| title_short | Multimodal Imaging and Biomarkers in Cardiac Amyloidosis |
| title_sort | multimodal imaging and biomarkers in cardiac amyloidosis |
| topic | amyloidosis immunoglobulin light chain amyloidosis transthyretin amyloidosis multimodal imaging biomarkers |
| url | https://www.mdpi.com/2075-4418/12/3/627 |
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