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Cardiac Amyloidosis
Sanjeev A. Francis, MD, FACC; Suhny Abbara, MD, FACR, FSCCT; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Amyloidosis: Heterogeneous group of diseases characterized by extracellular accumulation of abnormal fibrillar protein deposits (amyloid)
              • Cardiac involvement can result in infiltrative cardiomyopathy

            IMAGING

            • General Features

              • Echocardiographic Findings

                • Nuclear Medicine Findings

                  • CT Findings

                    • MR Findings

                      • Imaging Recommendations

                        • CT Findings

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              Selected References

                                              1. Falk RH: Tafamidis for transthyretin amyloid cardiomyopathy: the solution or just the beginning of the end? Eur Heart J. ePub, 2019
                                              2. Tang CX et al: Cardiovascular magnetic resonance imaging for amyloidosis: the state-of-the-art. Trends Cardiovasc Med. 29(2):83-94, 2019
                                              3. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                              4. Banypersad SM et al: T1 mapping and survival in systemic light-chain amyloidosis. Eur Heart J. 36(4):244-51, 2015
                                              5. Fontana M et al: Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis. Circulation. 132(16):1570-9, 2015
                                              6. Treibel TA et al: Extracellular volume quantification by dynamic equilibrium cardiac computed tomography in cardiac amyloidosis. J Cardiovasc Comput Tomogr. 9(6):585-92, 2015
                                              7. Dungu JN et al: CMR-based differentiation of AL and ATTR cardiac amyloidosis. JACC Cardiovasc Imaging. 7(2):133-42, 2014
                                              8. Falk RH et al: How to image cardiac amyloidosis. Circ Cardiovasc Imaging. 7(3):552-62, 2014
                                              9. White JA et al: CMR imaging with rapid visual T1 assessment predicts mortality in patients suspected of cardiac amyloidosis. JACC Cardiovasc Imaging. 7(2):143-56, 2014
                                              10. Bokhari S et al: (99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses. Circ Cardiovasc Imaging. 6(2):195-201, 2013
                                              11. Karamitsos TD et al: Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis. JACC Cardiovasc Imaging. 6(4):488-97, 2013
                                              12. Mongeon FP et al: Quantification of extracellular matrix expansion by CMR in infiltrative heart disease. JACC Cardiovasc Imaging. 5(9):897-907, 2012
                                              13. Wassmuth R et al: Prognostic impact of T2-weighted CMR imaging for cardiac amyloidosis. Eur Radiol. 21(8):1643-50, 2011
                                              14. Austin BA et al: Delayed hyper-enhancement magnetic resonance imaging provides incremental diagnostic and prognostic utility in suspected cardiac amyloidosis. JACC Cardiovasc Imaging. 2(12):1369-77, 2009
                                              15. Vogelsberg H et al: Cardiovascular magnetic resonance in clinically suspected cardiac amyloidosis: noninvasive imaging compared to endomyocardial biopsy. J Am Coll Cardiol. 51(10):1022-30, 2008
                                              16. vanden Driesen RI et al: MR findings in cardiac amyloidosis. AJR Am J Roentgenol. 186(6):1682-5, 2006
                                              17. Falk RH: Diagnosis and management of the cardiac amyloidoses. Circulation. 112(13):2047-60, 2005
                                              18. Kwong RY et al: Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 111(2):122-4, 2005
                                              19. Maceira AM et al: Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 111(2):186-93, 2005
                                              20. Dispenzieri A et al: Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 22(18):3751-7, 2004
                                              21. Ikeda S: Cardiac amyloidosis: heterogenous pathogenic backgrounds. Intern Med. 43(12):1107-14, 2004
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Definitions

                                                          • Amyloidosis: Heterogeneous group of diseases characterized by extracellular accumulation of abnormal fibrillar protein deposits (amyloid)
                                                          • Cardiac involvement can result in infiltrative cardiomyopathy

                                                        IMAGING

                                                        • General Features

                                                          • Echocardiographic Findings

                                                            • Nuclear Medicine Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Imaging Recommendations

                                                                    • CT Findings

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          Selected References

                                                                                          1. Falk RH: Tafamidis for transthyretin amyloid cardiomyopathy: the solution or just the beginning of the end? Eur Heart J. ePub, 2019
                                                                                          2. Tang CX et al: Cardiovascular magnetic resonance imaging for amyloidosis: the state-of-the-art. Trends Cardiovasc Med. 29(2):83-94, 2019
                                                                                          3. Rajiah P et al: Update on the role of cardiac magnetic resonance in acquired nonischemic cardiomyopathies. J Thorac Imaging. 31(6):348-66, 2016
                                                                                          4. Banypersad SM et al: T1 mapping and survival in systemic light-chain amyloidosis. Eur Heart J. 36(4):244-51, 2015
                                                                                          5. Fontana M et al: Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis. Circulation. 132(16):1570-9, 2015
                                                                                          6. Treibel TA et al: Extracellular volume quantification by dynamic equilibrium cardiac computed tomography in cardiac amyloidosis. J Cardiovasc Comput Tomogr. 9(6):585-92, 2015
                                                                                          7. Dungu JN et al: CMR-based differentiation of AL and ATTR cardiac amyloidosis. JACC Cardiovasc Imaging. 7(2):133-42, 2014
                                                                                          8. Falk RH et al: How to image cardiac amyloidosis. Circ Cardiovasc Imaging. 7(3):552-62, 2014
                                                                                          9. White JA et al: CMR imaging with rapid visual T1 assessment predicts mortality in patients suspected of cardiac amyloidosis. JACC Cardiovasc Imaging. 7(2):143-56, 2014
                                                                                          10. Bokhari S et al: (99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses. Circ Cardiovasc Imaging. 6(2):195-201, 2013
                                                                                          11. Karamitsos TD et al: Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis. JACC Cardiovasc Imaging. 6(4):488-97, 2013
                                                                                          12. Mongeon FP et al: Quantification of extracellular matrix expansion by CMR in infiltrative heart disease. JACC Cardiovasc Imaging. 5(9):897-907, 2012
                                                                                          13. Wassmuth R et al: Prognostic impact of T2-weighted CMR imaging for cardiac amyloidosis. Eur Radiol. 21(8):1643-50, 2011
                                                                                          14. Austin BA et al: Delayed hyper-enhancement magnetic resonance imaging provides incremental diagnostic and prognostic utility in suspected cardiac amyloidosis. JACC Cardiovasc Imaging. 2(12):1369-77, 2009
                                                                                          15. Vogelsberg H et al: Cardiovascular magnetic resonance in clinically suspected cardiac amyloidosis: noninvasive imaging compared to endomyocardial biopsy. J Am Coll Cardiol. 51(10):1022-30, 2008
                                                                                          16. vanden Driesen RI et al: MR findings in cardiac amyloidosis. AJR Am J Roentgenol. 186(6):1682-5, 2006
                                                                                          17. Falk RH: Diagnosis and management of the cardiac amyloidoses. Circulation. 112(13):2047-60, 2005
                                                                                          18. Kwong RY et al: Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 111(2):122-4, 2005
                                                                                          19. Maceira AM et al: Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 111(2):186-93, 2005
                                                                                          20. Dispenzieri A et al: Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 22(18):3751-7, 2004
                                                                                          21. Ikeda S: Cardiac amyloidosis: heterogenous pathogenic backgrounds. Intern Med. 43(12):1107-14, 2004