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Page 4 of 11                 Mattana et al. Vessel Plus 2022;6:13  https://dx.doi.org/10.20517/2574-1209.2021.87






























                Figure 3. Technetium-99m 3,3-diphosphono-1,2-propanodicarboxylic acid bone scan, 3 h acquisition: (A) visual score 0; (B) visual
                score 1 and corresponding single photon emission computed tomography/computed tomography image; (C) visual score 2; and (D)
                visual score 3.

               Despite the high diagnostic accuracy reported in the literature, CA remains underdiagnosed and sometimes
               misdiagnosed. The most common and important cause of misdiagnosis is the failure to exclude AL
               cardiomyopathy (AL-CM) in patients with high tracer uptake (Grade 2 or 3 on planar images). Although in
               the literature myocardial uptake in AL-CM is usually described as Grade 1 or less, larger cohorts reported
               that 22% of patients showing Grade 2 or 3 uptakes on  Tc-DPD or  Tc-PYP scans have AL-CM on the
                                                              99m
                                                                          99m
               EMB. Other cases of misdiagnosis can be related to some pitfalls due to a wrong imaging interpretation
               and/or an uncorrected acquisition protocol.

               Despite the emphasis in published guidelines, failure or incomplete screening for AL amyloidosis has been
               reported in up to 24% of patients undergoing  Tc-PYP ; in addition, many centers only perform planar
                                                               [20]
                                                      99m
               imaging acquisition (without SPECT imaging to confirm myocardial uptake), with the high risk of
               misinterpreting the blood pool uptake as myocardial uptake. In this paper, we aim to briefly describe the
               main differences between  Tc-PYP and  Tc-DPD, analyzing the data available in the literature and
                                      99m
                                                    99m
               highlighting the most frequent causes of misdiagnosis and pitfalls.

               ACQUISITION PROTOCOLS AND DIAGNOSTIC CRITERIA
               99m
                 Tc-PYP
               This tracer is the only used tracer in the United States. Nevertheless, a standardized and shared acquisition
               protocol is still lacking  so it is difficult to correctly compare and analyze the data from the different
                                   [21]
                                                                                                       [22]
               studies. The American Society of Nuclear Cardiology (ASNC) released the latest Practice Points in 2019 ,
                                        99m
               suggesting to standardize the  Tc-PYP acquisition protocol. The writing committee recommended planar
               imaging followed by the single-photon emission computerized tomography (SPECT) imaging or
               SPECT/CT imaging at 1 h; an optional SPECT imaging at 3 h can be used when a high blood pool activity
               has been observed in the 1 h SPECT images. The semi-quantitative analysis criteria reckon on a heart to
               contralateral lung ratio (H/CL) > 1.5 on 1 h imaging to accurately identify ATTR cardiac amyloidosis if
               systemic AL amyloidosis has been excluded. A similar recommendation was reported in a multi-societal
               expert consensus on imaging CA .
                                           [23]
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