Nuclear Molecular Imaging of Disease Burden and Response to Treatment for Cardiac Amyloidosis

SIMPLE SUMMARY: Cardiac amyloidosis (CA) is characterized by extracellular infiltration and deposition of amyloid fibrils primarily derived from the circulating transthyretin protein (TTR) or immunoglobulin light chain (AL). With the development of non-invasive diagnostic approaches and the emergence of new pharmacotherapeutic treatments for CA, the transformative effects of bone scintigraphy have been important in diagnosing TTR-CA. Positron emission tomography (PET) imaging is another promising, non-invasive option for the diagnosis of CA and may help differentiate between ATTR and AL amyloidosis. Bone-seeking single-photon emission tomography/computed tomography (SPECT/CT) quantification and amyloid-targeting PET imaging could be useful as a new strategy for disease burden and therapy monitoring to provide more insights into therapy response assessed by quantifying the amyloid burden in CA. ABSTRACT: Cardiac amyloidosis (CA) is a heterogeneous group of diseases in which extracellular insoluble amyloid proteins are deposited in specific organs and tissues locally or systemically, thereby interfering with physiological function. Transthyretin protein (TTR) and light chain (AL) amyloidosis are the most common types of cardiac amyloidosis. Radionuclide bone scintigraphy has recently become the most common non-invasive test for the diagnosis of TTR-CA but is of limited value for the diagnosis of AL-CA. PET has proved promising for the diagnosis of CA and its applications are expected to expand in the future. This review summarizes the current bone scintigraphy and amyloid-targeting Positron emission tomography (PET) imaging, the binding imaging properties of radiotracers, and the values of diagnosis, prognosis, and monitoring therapy response in CA.