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EACVI Survey on Capturing current practices in cardiac amyloid suspicion, diagnosis and management across Europe by multimodality Imaging

Cardiac amyloidosis is increasingly recognised as an important and potentially treatable cause of cardiomyopathy, with accurate differentiation between AL (immunoglobulin light-chain) and ATTR (transthyretin) amyloidosis being essential due to differences in prognosis and treatment. This EACVI survey aims to evaluate current European practices in multimodality imaging for the suspicion, diagnosis, and management of cardiac amyloidosis. By assessing imaging pathways, diagnostic resources, and multidisciplinary collaboration, the survey seeks to identify gaps in practice, support future EACVI recommendations, and guide educational initiatives to improve imaging-led care across Europe.
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• The survey is anonymous
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1.What is your age range?
2.What is your gender?
3.Your role (Single answer)
4.Country
5.Which type of centre are you currently working at?
6.Which imaging modality is your main area of expertise? (May select more than one option)
7.Which diagnostic modalities are routinely available at your institution, outside genetic testing? (May select more than one option)
8.Which of these applications are routinely available at your institution? (May select more than one option)
9.On a personal level, how many cases do you manage for cardiac amyloidosis per year?
10.How many new diagnoses or suspected cases of cardiac amyloidosis are seen per year at your centre?
11.How are new cardiac amyloidosis cases managed at your institution? (Single answer)
12.In your opinion, what are the main phenocopies/differentials when referring for suspected cardiac amyloidosis? Please rate from 1 (least challenging to differentiate) to 5 (most challenging)
1 (least challenging to differentiate)
2
3
4
5 (most challenging)
Hypertensive heart disease
Hypertrophic cardiomyopathy
Calcific aortic valve stenosis
Fabry’s or other storage diseases
Other causes of restrictive cardiomyopathy (RCM)
13.How often do you perform imaging-based screening for cardiac amyloidosis in the following at-risk subgroups?
rarely or never
selectively
systematically
HFpEF patients (heart failure with preserved ejection fraction)
Elderly patients with aortic stenosis
Unexplained ventricular hypertrophy patients
Patients with bilateral carpal tunnel syndrome or spinal stenosis and cardiac symptoms
Monoclonal gammopathy patients with cardiac abnormalities
14.In your daily practice which are the most important echocardiographic findings raising suspicion of cardiac amyloidosis?
1 (Least important)
2
3
4
5 (Most important)
Concentric LV Hypertrophy with speckle appearance
Concentric LV Hypertrophy with advanced diastolic dysfunction
Concentric LV and RV hypertrophy and pericardial effusion
Apical sparing on longitudinal strain
Biatrial enlargement, diastolic dysfunction with thickened valves and intra-atrial septum
15.Which of the following echocardiographic measurements would you consider to track disease progression or regression post disease-modifying therapy? (May select more than one option)
16.Which CMR findings are most influential in your diagnosis of cardiac amyloidosis? (Select up to three)
17.How often do you use the following CMR parameters to assess disease progression or treatment response in your daily practice?
rarely
selectively
systematically
Concentric LV and RV hypertrophy and pericardial effusion
Abnormal myocardial nulling pattern on late gadolinium enhancement
Diffuse subendocardial or transmural LGE patterns
Native T1 mapping values and pattern
Extracellular volume (ECV) expansion
18.When do you use bone-scintigraphy imaging in your practice as part of the diagnostics in suspected cardiac amyloidosis?
19.In your practice, for which of the following purposes do you use bone scintigraphy in the evaluation of suspected or confirmed cardiac amyloidosis? (May select more than one option)
20.How often do you encounter operational barriers at your centre for the diagnosis of cardiac amyloidosis?
Never or almost never encounter this barrier.
Rarely encounter this barrier.
Often encounter this barrier.
Lack of availability of certain imaging modalities
Long waiting times
Inconclusive results and interpretation challenges
Cost for the institution
Patient-related factors (e.g. renal failure, frailty, etc.)
21.Which developments do you believe would have the greatest impact on improving the field of cardiac amyloidosis?
22.At your centre, what is the estimated time from cardiac amyloidosis suspicion to final diagnosis, including all imaging and amyloid subtyping?
23.What is your usual next step when a patient has Perugini grade 1 intramyocardial uptake on bone-scintigraphy?
24.If a patient has an extra-cardiac amyloid-positive tissue biopsy, what would you minimally require to establish cardiac amyloidosis involvement?
25.According to your experience, what is the estimated percentage of cardiac amyloidosis due to the incidental finding of cardiac uptake on bone scintigraphy?