ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 1 of 2—evidence base and standardized methods of imaging

Abstract

accurate recognition at earlier stages. Imaging provides non-invasive tools for follow-up of disease remission/progression complementing clinical evaluation. Additional areas not defined include appropriate clinical indications for imaging, optimal imaging utilization by clinical presentation, accepted imaging methods, accurate image interpretation, and comprehensive and clear reporting. Prospective randomized clinical trial data for the diagnosis of amyloidosis and for imaging-based strategies for treatment are not available. A consensus of expert opinion is greatly needed to guide the appropriate clinical utilization of imaging in cardiac amyloidosis.

Reversible myocardial oedema due to acute myocardial infarction as differential diagnosis of cardiac transthyretin amyloidosis

“…11 The described phenomenon, that not only cardiac TTR amyloidosis but also acute myocardial infarction, especially after reperfusion, may lead to positive technetium-99m phosphorus scans, is only sporadically mentioned in recent review articles. 4,12 To differentiate between amyloidosis and myocardial infarction, it is suggested to obtain information whether the uptake is regional-typical for ischaemia-or diffuse-typical for TTR amyloidosis. 4,12 In the presented patient, however, only projections in the anterior/posterior view had been performed; thus, no information about the regional tracer distribution is available.…”

“…4,12 To differentiate between amyloidosis and myocardial infarction, it is suggested to obtain information whether the uptake is regional-typical for ischaemia-or diffuse-typical for TTR amyloidosis. 4,12 In the presented patient, however, only projections in the anterior/posterior view had been performed; thus, no information about the regional tracer distribution is available.…”

“…5,6 Granular sparkling is an echocardiographic finding that is not specific for amyloidosis but has also been reported in end-stage renal disease, hypertrophic cardiomyopathy, and Friedreich cardiomyopathy. 4,14,15 Myocardial thickening, furthermore, may be due to many different conditions and diseases. 15,16 In the case presented, inappropriate testing and interpretation of the images resulted in an Figure 2 99m-Tc DPD/HDP bone scintigraphy 4 days after myocardial infarction shows a myocardial tracer uptake Perugini Score 2 (blue arrow) (A).…”

“…1,2 Radionuclide imaging with bone-avid radiotracers is reported to specifically detect TTR cardiac amyloidosis, thus obviating the need for endomyocardial biopsy to establish the diagnosis. 1,3,4 The 2017 consensus document of the European Association of Cardiovascular Imaging (EACVI) and the Working Group on myocardial and pericardial diseases of the European Society of Cardiology (ESC) on multimodality imaging in restrictive cardiomyopathy states that the diagnosis of cardiac TTR amyloidosis can reliably be made by 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99m-Tc DPD) scintigraphy without the need for histology. 3 Similar recommendations from American and Japanese authors are given in recently published review articles.…”

What is the optimal cardiac screening for asymptomatic transthyretin gene mutation carriers?

“…Recently, expert consensus recommendations have been announced by multiple societies. 12,13 The recommendations categorized repeated bone scintigraphy to be appropriate in the screening of asymptomatic TTR gene mutation carriers, however, the indications and suggested interval were not addressed. In this study, among the patients without cardiac uptake on the baseline Tc-99m DPD scan, some showed positive cardiac uptake at the 2-year follow-up scan (2 out of 8; 25%).…”