Quantification of amyloid deposition using bone scan agents
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Department of Radioisotope Medicine, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan Department of Radioisotope Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
Received Aug 7, 2020; accepted Aug 7, 2020 doi:10.1007/s12350-020-02340-9
See related article, doi:https://doi.org/10. 1007/s12350-020-02274-2 CARDIAC AMYLOIDOSIS: EMERGING REQUIREMENTS FOR AN ACCURATE DIAGNOSIS Cardiac amyloidosis is one of the most underdiagnosed cardiovascular disease entities. It was previously considered to be a rare condition, but was recently identified as one of the main causes of heart failure, particularly HFpHF (heart failure with preserved ejection fraction), in elderly patients.1 There are several subtypes of amyloid, with major cardiac effects being AL (light-chain) and ATTR (transthyretin) amyloidosis. AL amyloidosis occurs as a consequence of immunoglobulin-related diseases, such as multiple myeloma, and has a very poor prognosis. ATTR amyloidosis is subdivided into wild and mutant types. The mutant type occurs infrequently with genetic changes and a clear family history. In contrast, wild-type ATTR amyloidosis, also known as senile systemic amyloidosis (SSA), is caused by the deposition of wild-type ATTR amyloid on multiple organs, including the myocardium, and is common in elderly patients. A population-based study conducted in Finland2 revealed a strikingly high prevalence of the deposition of ATTR in very old (over 85) subjects (25%). The difficulties associated with diagnosing cardiac amyloidosis have resulted in its underdiagnosis. The
Reprint requests: Takashi Kudo, MD, PhD, Department of Radioisotope Medicine, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki, Japan; [email protected] J Nucl Cardiol 1071-3581/$34.00 Copyright Ó 2020 American Society of Nuclear Cardiology.
classical ‘‘stigmata’’ symptoms of systemic amyloidosis include ‘‘panda eyes’’, macroglossia, carpal tunnel symptoms, and nail dystrophy. However, patients with ATTR amyloidosis may exhibit few of the characteristic symptoms and signs of this disease, which may be overlooked when ATTR amyloidosis is not one of the diagnostic options in the attending clinician’s mind. However, the importance of this disease is now being recognized, and many cardiologists consider cardiac amyloidosis as one of the causes of unexplained heart failure in the elderly. Since invasive biopsy is required for the final diagnosis of cardiac amyloidosis, the development of non-invasive diagnostic procedures is needed. Several non-invasive methods, such as apical sparing in echocardiography3 and T1 mapping in CMR,4 have good diagnostic accuracy. However, the findings of these methods are not directly related to the molecular etiology of cardiac amyloidosis. The background of nuclear cardiology imaging involves molecular imaging and provides a direct link between the findings obtained and etiology. Three nuclear medicine imaging techniques are performed in clinical settings: planar, SPECT, and PET
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