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
Bokhari S et al: Standardization of 99mTechnetium pyrophosphate imaging methodology to diagnose TTR cardiac amyloidosis. J Nucl Cardiol. 25(1):181-90, 2018
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
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
Bokhari S et al: Nuclear imaging modalities for cardiac amyloidosis. J Nucl Cardiol. 21(1):175-84, 2014
Related Anatomy
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Related Differential Diagnoses
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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
Bokhari S et al: Standardization of 99mTechnetium pyrophosphate imaging methodology to diagnose TTR cardiac amyloidosis. J Nucl Cardiol. 25(1):181-90, 2018
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
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
Bokhari S et al: Nuclear imaging modalities for cardiac amyloidosis. J Nucl Cardiol. 21(1):175-84, 2014
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