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PROGRESSION IS FAST,
TIMELY DIAGNOSIS IS KEY1–3

UNDERSTANDING THE DIAGNOSTIC
ALGORITHM FOR ATTR-CM

Speaker presenting on stage at a TTR Masterclass medical conference

In this video, Dr Garcia-Pavia discusses the role of scintigraphy in diagnosing ATTR-CM, as well as how to interpret other diagnostic screening tests for ATTR-CM.

SEEING ANY OF THE INVESTIGATIVE
FINDINGS BELOW, AND ESPECIALLY IN
COMBINATION, SHOULD TRIGGER
ATTR-CM CONFIRMATORY TESTS1,3,4

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ECG4
  • Pseudo-infarction pattern
  • QRS voltages disproportionate to LV wall thickness
  • Atrioventricular block in the presence of LVH

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ECHO4
  • Hypertrophic phenotype with infiltrative features
  • Restrictive LV filling with RV wall thickening
  • Low septal and lateral e′
  • Reduction in global longitudinal strain

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c-MRI4
  • Increased ECV
  • Diffuse LGE

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Proactively looking for these findings in regular clinical practice may facilitate an ATTR-CM diagnosis5

Once ATTR-CM is suspected, ACT FAST. Learn about the diagnostic process to confirm ATTR-CM

DIAGNOSE ATTR-CM IN
TWO KEY STEPS:6

illustrating serum free light chain testing used for excluding AL amyloidosis

FIND OUT WHAT TO LOOK FOR IN MONOCLONAL PROTEIN SCREENING large copy link icon large copy link on hover icon 

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the use of scintigraphy to confirm an ATTR-CM diagnosis

FIND OUT WHAT TO LOOK FOR IN SCINTIGRAPHY large copy link icon large copy link on hover icon 

HOW TO REACH A DIAGNOSIS
OF ATTR-CM6

Flowchart showing diagnostic steps for ATTR-CM using scintigraphy, light‑chain testing, AL exclusion, and biopsy

*99mTc-HMDP scintigraphy can also be used.6

†Histological confirmation of amyloid deposits (can be cardiac or extracardiac) is required. If the biopsy is extracardiac, and accompanied with characteristic features on ECHO or c-MRI, diagnosis can be confirmed. Cardiac amyloidosis on biopsy is characterised by extracellular deposition of misfolded proteins with pathognomonic histological property of green birefringence when viewed under cross-polarised light after staining with Congo red.6

ONCE ATTR-CM HAS BEEN CONFIRMED,
PERFORM GENETIC TESTING TO
DIFFERENTIATE BETWEEN HEREDITARY
AND WILD-TYPE DISEASE6

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hATTR and wtATTR can present differently, and may require a

different treatment and management strategy1,6 

 
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Detection of a pathogenic TTR variant allows for

testing of family members

and earlier detection of symptoms and treatment intervention7,8

ONCE ATTR-CM IS CONFIRMED, ACT FAST

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TREAT

Specific ATTR-CM treatments are available.9 Initiating these treatments as early as possible can lead to improved outcomes9

[Local information for guidance on treating ATTR-CM]

 

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OR REFER

ATTR-CM is a rapidly progressive disease.4 It is vital to ACT FAST. Referral to a specialist centre may ensure patients get the right treatment, sooner

[Local information for guidance on treating ATTR-CM]

 

Once ATTR-CM is confirmed, ACT FAST. Learn about how patients may present in your clinic

 
FIND OUT WHERE YOU CAN REFER YOUR PATIENTS