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Cardiac Amyloidosis
Fathima Fijula P. Manzil, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Diagnostic Checklist

        TERMINOLOGY

        • Definitions

          • Cardiac amyloidosis: Abnormal beta-pleated sheets of protein infiltrating heart
            • Most (> 95%) caused by amyloid light chain (AL) or amyloid transthyretin (ATTR)
            • AL amyloidosis 
              • Plasma cell disorder with overproduction of lambda and kappa light chains that form amyloid deposits; treated with chemotherapy (similar to multiple myeloma, another plasma cell disorder)
            • ATTR amyloidosis
              • Transthyretin protein amyloid deposits; treated with heart transplant when advanced
              • ATTRm (mutant) variant: Point mutations in TTR
              • ATTRwt (wild-type) variant: No TTR mutation
            • Other amyloid types involving heart: Amyloid A, apolipoprotein AI, heavy chain, atrial natriuretic peptide
            • Endomyocardial biopsy gold standard for diagnosis

        IMAGING

        • Nuclear Medicine Findings

          • Imaging Recommendations

            DIFFERENTIAL DIAGNOSIS

              CLINICAL ISSUES

              • Presentation

                • Demographics

                  • Natural History & Prognosis

                    • Treatment

                      DIAGNOSTIC CHECKLIST

                      • Image Interpretation Pearls

                        Selected References

                        1. Bokhari S et al: Standardization of 99mTechnetium pyrophosphate imaging methodology to diagnose TTR cardiac amyloidosis. J Nucl Cardiol. 25(1):181-90, 2018
                        2. Harb SC et al: National patterns in imaging utilization for diagnosis of cardiac amyloidosis: A focus on Tc99m-pyrophosphate scintigraphy. J Nucl Cardiol. 24(3):1094-1097, 2017
                        3. 99mTechnetium-Pyrophosphate Imaging for Transthyretin Cardiac Amyloidosis. ASNC Practice Points. https://www.asnc.org/Files/Practice%20Resources/Practice%20Points/ASNC%20Practice%20Point-99mTechnetiumPyrophosphateImaging2016.pdf. Published 2016. Accessed July 3, 2019
                        4. Bokhari S et al: Nuclear imaging modalities for cardiac amyloidosis. J Nucl Cardiol. 21(1):175-84, 2014
                        Related Anatomy
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                        References
                        Tables

                        Tables

                        KEY FACTS

                        • Terminology

                          • Imaging

                            • Diagnostic Checklist

                              TERMINOLOGY

                              • Definitions

                                • Cardiac amyloidosis: Abnormal beta-pleated sheets of protein infiltrating heart
                                  • Most (> 95%) caused by amyloid light chain (AL) or amyloid transthyretin (ATTR)
                                  • AL amyloidosis 
                                    • Plasma cell disorder with overproduction of lambda and kappa light chains that form amyloid deposits; treated with chemotherapy (similar to multiple myeloma, another plasma cell disorder)
                                  • ATTR amyloidosis
                                    • Transthyretin protein amyloid deposits; treated with heart transplant when advanced
                                    • ATTRm (mutant) variant: Point mutations in TTR
                                    • ATTRwt (wild-type) variant: No TTR mutation
                                  • Other amyloid types involving heart: Amyloid A, apolipoprotein AI, heavy chain, atrial natriuretic peptide
                                  • Endomyocardial biopsy gold standard for diagnosis

                              IMAGING

                              • Nuclear Medicine Findings

                                • Imaging Recommendations

                                  DIFFERENTIAL DIAGNOSIS

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Bokhari S et al: Standardization of 99mTechnetium pyrophosphate imaging methodology to diagnose TTR cardiac amyloidosis. J Nucl Cardiol. 25(1):181-90, 2018
                                              2. Harb SC et al: National patterns in imaging utilization for diagnosis of cardiac amyloidosis: A focus on Tc99m-pyrophosphate scintigraphy. J Nucl Cardiol. 24(3):1094-1097, 2017
                                              3. 99mTechnetium-Pyrophosphate Imaging for Transthyretin Cardiac Amyloidosis. ASNC Practice Points. https://www.asnc.org/Files/Practice%20Resources/Practice%20Points/ASNC%20Practice%20Point-99mTechnetiumPyrophosphateImaging2016.pdf. Published 2016. Accessed July 3, 2019
                                              4. Bokhari S et al: Nuclear imaging modalities for cardiac amyloidosis. J Nucl Cardiol. 21(1):175-84, 2014