A DIAGNOSTIC APPROACH FOR PATIENTS WITH SUSPECTED CARDIAC AMYLOIDOSIS THAT INCLUDES TESTING FOR MONOCLONAL PROTEIN FOLLOWED BY SCINTIGRAPHY AND/OR BIOPSY 1 Reprinted with permission from Maurer MS, Bokhari S, Damy T, et al. Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis. Circ Heart Fail . 2019;12:e006075. doi:10.1161/CIRCHEARTFAILURE.119.006075 © 2019 American Heart Association, Inc. All rights reserved. • 99m Technetium-pyrophosphate ( 99m Tc-PYP) is a noninvasive radioactive tracer utilized as an adjunct in the diagnosis of ATTR-CM, though not FDA approved for that use † • Both planar and SPECT imaging should be reviewed and interpreted using visual and quantitative approaches 1 *If fat pad is negative, biopsy of involved organ is required. † Please consult individual labeling for risks. ATTRm, mutant transthyretin amyloidosis; MGUS, monoclonal gammopathy of undetermined significance; MRI, magnetic resonance imaging; SPECT, single-photon emission computed tomography. Reference: 1. Maurer MS, Bokhari S, Damy T, et al. Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis. Circ Heart Fail . 2019;12:e006075. doi:10.1161/CIRCHEARTFAILURE.119.006075 © 2021 Pfizer Inc. All rights reserved. January 2021 PP-R1D-USA-0839-01 Referral to Hematology AL, ATTR, other amyloidosis and/or MGUS 1 or more abnormal all normal Non-invasive evaluation with Bone Scintigraphy • Positive Bone Scintigraphy Referral for Bone Scintigraphy or invasive evaluation with Heart Biopsy • Positive Congo Red • Tissue typing by mass spectrometry or immunostaining Cardiac Amyloidosis Unlikely Cardiac Amyloidosis Unlikely Consider Heart Biopsy if suspicion is high Cardiac Amyloidosis Unlikely yes no yes Negative or indeterminate ATTRwt ATTRm ATTR Amyloidosis yes yes no no Genetic Testing Screen for the presence of a monoclonal protein Order the following three tests: • Serum kappa/lambda free light chain ratio (abnormal if ratio is <0.26 or >1.65) • Serum protein immunofixation (abnormal if monoclonal protein is detected) • Urine protein immunofixation (abnormal if monoclonal protein is detected) Symptoms, ECG, Echo, MRI, or Biomarkers suggestive of cardiac amyloidosis Biopsy of clinically involved organ (cardiac or renal) or fat pad* • Positive Congo Red • Tissue typing by mass spectrometry of immunostaining Bone Scintigraphy Available? ATTRwt ATTRm ATTR Amyloidosis Genetic Testing
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A D I A G N O S T I C A P P R O A C HF O R PAT I E N T S W I T H S U S P E C T E D C A R D I A C A M Y L O I D O S I S
T H AT I N C L U D E S T E S T I N G F O R M O N O C L O N A L P R O T E I N F O L L O W E D B Y S C I N T I G R A P H Y A N D / O R B I O P S Y 1
Reprinted with permission from Maurer MS, Bokhari S, Damy T, et al. Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis. Circ HeartFail. 2019;12:e006075. doi:10.1161/CIRCHEARTFAILURE.119.006075 © 2019 American Heart Association, Inc. All rights reserved.
• 99mTechnetium-pyrophosphate (99mTc-PYP) is a noninvasive radioactive tracer uti l ized as an adjunct in the diagnosis of ATTR-CM, though not FDA approved for that use†
• Both planar and SPECT imaging should be reviewed and interpreted using visual and quantitative approaches1
*If fat pad is negative, biopsy of involved organ is required.†Please consult individual labeling for risks.
ATTRm, mutant transthyretin amyloidosis; MGUS, monoclonal gammopathy of undetermined significance; MRI, magnetic resonance imaging; SPECT, single-photon emission computed tomography.
Reference: 1. Maurer MS, Bokhari S, Damy T, et al. Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis. Circ Heart Fail. 2019;12:e006075. doi:10.1161/CIRCHEARTFAILURE.119.006075
© 2021 Pfizer Inc. All r ights reserved. January 2021 PP-R1D-USA-0839-01
Referral to Hematology
AL, ATTR, other amyloidosis and/or
MGUS
1 or more abnormal all normal
Non-invasive evaluation with Bone Scintigraphy• Positive Bone Scintigraphy
Referral for Bone Scintigraphy or invasive evaluation with Heart Biopsy• Positive Congo Red • Tissue typing by mass
spectrometry or immunostaining
Cardiac Amyloidosis
Unlikely
Cardiac Amyloidosis
Unlikely
Consider Heart Biopsy if
suspicion is high
Cardiac Amyloidosis
Unlikely
yes noyes Negative or
indeterminate
ATTRwt ATTRm
ATTR Amyloidosis
yes
yes
no
no
Genetic Testing
Screen for the presence of a monoclonal proteinOrder the following three tests:
• Serum kappa/lambda free light chain ratio (abnormal if ratio is <0.26 or >1.65)• Serum protein immunofixation (abnormal if monoclonal protein is detected)• Urine protein immunofixation (abnormal if monoclonal protein is detected)
Symptoms, ECG, Echo, MRI, or Biomarkers suggestive of cardiac amyloidosis
Biopsy of clinically involved organ (cardiac or renal) or fat pad*
• Positive Congo Red • Tissue typing by mass spectrometry of
immunostaining
Bone Scintigraphy Available?
ATTRwt ATTRm
ATTR Amyloidosis
Genetic Testing
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