Abstract
PurposeHereditary transthyretin-amyloid amyloidosis (ATTRv) is an underdiagnosed condition commonly manifesting as congestive heart failure. Recently, scintigraphy utilizing DPD as a tracer was shown to identify ATTRv and wild-type ATTR cardiomyopathy. The aim of this study was to determine the value of quantified scintigraphy utilizing 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) single-photon emission computed tomography (SPECT)/CT, and to correlate its uptake with well-established cardiac functional parameters.MethodsForty-eight patients with genetically verified ATTRv type-A fibril composition, positive 99mTc-DPD SPECT/CT, were retrospectively analyzed. Manual mapping of volumes of interest (VOIs) on DPD SPECT/CT examinations was used to quantify heart uptake. DPD mean and maximum uptake together with a calculated DPD-based amyloid burden (DPDload) was correlated with echocardiographic strain values and cardiac biomarkers.ResultsStatistically significant correlations were seen in VOIs between DPD uptakes and the corresponding echocardiographic strain values. Furthermore, DPDload had a strong correlation with echocardiographic strain parameters and also correlated with biomarkers troponin T and logarithmic NT-ProBNP.ConclusionsIn patients with ATTRv cardiomyopathy, DPD SPECT/CT measures the amyloid distribution and provides information on cardiac amyloid load. DPD amyloid load correlates with functional cardiac parameters.
Highlights
Systemic amyloidosis is a group of diseases caused by the deposition of insoluble fibrils, known as amyloid fibrils, in the extracellular spaces of tissues
Status of liver transplantation, and visual scoring grade on DPD uptake according to Perugini grading was acquired; the latter was performed in a clinical setting
A relative apical sparing was calculated according to the following formula: average apical longitudinal strain/(average basal longitudinal strain + average midlongitudinal strain), with a cut-off of 1 [40]
Summary
Systemic amyloidosis is a group of diseases caused by the deposition of insoluble fibrils, known as amyloid fibrils, in the extracellular spaces of tissues. One of the most common causes of CA is transthyretin (TTR) amyloid (ATTR) amyloidosis [6]. ATTR is a heterogeneous disease that can be divided into a hereditary form caused by mutations in the transthyretin-gene (ATTRv) and wild-type transthyretin amyloidosis (ATTRwt)—previously designated senile systemic amyloidosis [7]. It is of importance to identify ATTR-CA as conventional heart failure medication has no proven effect on prognosis and might even be harmful [8,9,10]. Nuclear medicine imaging by scintigraphy has become an important part of the diagnosis of ATTR CA. All regarded effective, have been employed including 99mTechnetium (Tc)-bisphosphonate
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
