It is difficult to say that there is more evidence for cardiac amyloidosis (CA) than for ischemic heart disease. On the other hand, 99 m technetium pyrophosphate ( 99 m Tc-PYP) scintigraphy has been reported to be useful with high sensitivity and specificity, especially in transthyretin (TTR) amyloidosis (ATTR) 1,2 Due to the spread of diagnosis using this method, CA, especially wild-type ATTR (ATTRwt) amyloidosis, which has traditionally been considered a rare disease, is more prevalent than previously assumed, and encountered relatively frequently in daily clinical practice. Furthermore, treatment for not only amyloid light-chain (AL) amyloidosis, but also ATTR, has also progressed rapidly. Tafamidis, a drug that stabilizes the TTR tetramer and suppresses amyloid fibril formation and tissue deposition, was listed and used in Japan in November 2013 for the treatment of peripheral neuropathy in patients with hereditary (variant) ATTR (ATTRv) amyloidosis. In addition, following the results of the 2018 Transthyretin Amyloidosis Cardiomyopathy Clinical Trial (ATTR-ACT), which showed the efficacy of tafamidis for CA, 3 the use of TTR to treat CA was approved in March 2019 in Japan.Needless to say, amyloidosis is a systemic disease, and in Japan, numerous studies have been conducted and medical treatments devised by the Ministry of Health, Labour and Welfare (MHLW)'s "Research Group on Amyloidosis", a research project on intractable disease policy. The present guidelines have been developed in coordination between the MHLW's "Research Group on Amyloidosis", the Japanese Circulation Society (JCS) and cardiology-related societies, the Japanese Society of Amyloidosis, and the Japanese Society of Hematology. Systemic amyloidosis specified by the MHLW as being an incurable disease is classified into the following four types.

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AimsAlthough previous reports suggest that an elevated endogenous erythropoietin (EPO) level is associated with worse clinical outcomes in chronic heart failure (HF) patients, the prognostic implication of EPO in patients with acute decompensated HF (ADHF) Methods and resultsWe examined 539 consecutive ADHF patients with EPO measurement on admission from our registry. During a median follow-up period of 329 days, a higher EPO level on admission was independently associated with worse clinical outcomes [hazard ratio (HR) 1.25, 95% confidence interval (CI) 1.06-1.48, P = 0.008], and haemoglobin level was the strongest determinant of EPO level (P < 0.001), whereas estimated glomerular filtration rate (eGFR) was not significant in multivariate regression analysis. In the anaemic subgroup of 318 patients, a higher EPO level than expected on the basis of their haemoglobin level was related to increased adverse events (HR 1.63, 95% CI 1.05-2.49, P = 0.028). Moreover, estimated plasma volume excess rate was positively associated with EPO level (P = 0.003), and anaemic patients with a higher than expected EPO level tended to have a higher estimated plasma volume excess rate and plasma lactate level, and lower systemic oxygen saturation level with the preservation of the reticulocyte production index than those with a lower than expected EPO level.

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Transthyretin amyloid cardiomyopathy (ATTR‐CM) is caused by the cardiac deposition of insoluble amyloid fibrils formed by misfolded transthyretin proteins and is associated with various cardiac symptoms, such as progressive heart failure, conduction disturbance, and arrhythmia. The implementation of 99mtechnetium (99mTc)‐labelled bone radiotracer scintigraphy for diagnosing ATTR‐CM has enabled accurate diagnosis of the disease with high sensitivity and specificity and positioned this diagnostic modality as an integral part of disease diagnostic algorithms. In 2020, 99mTc‐pyrophosphate scintigraphy received exceptional approval for Japanese national health insurance reimbursement as a diagnostic method of ATTR‐CM. Nevertheless, the utility of 99mTc‐labelled bone radiotracer scintigraphy and the importance of an early diagnosis of suspected ATTR‐CM using this technique have yet to be internalized as common practice by general cardiologists, and guidance on daily clinical scenarios to consider this technique for a diagnosis of suspected ATTR‐CM is warranted. In this review, we discuss the utility of 99mTc‐labelled bone radiotracer scintigraphy for the early diagnosis of ATTR‐CM based on published literature and the outcomes of an advisory board meeting. This review also discusses clinical scenarios that could support early diagnosis of suspected ATTR‐CM as well as common pitfalls, correct implementation, and future perspectives of 99mTc‐labelled bone radiotracer scintigraphy in daily clinical practice. The clinical scenarios to consider 99mTc‐labelled bone radiotracer scintigraphy in daily practice may include, but are not limited to, patients with a family history of the hereditary type of disease; elderly patients (aged ≥60 years) with unexplained cardiac findings (e.g. cardiac hypertrophy associated with abnormalities on an electrocardiogram, heart failure with preserved ejection fraction associated with unexplained left ventricular hypertrophy, and heart failure with reduced ejection fraction associated with atrial fibrillation and left ventricular hypertrophy); and patients with cardiac hypertrophy associated with diastolic dysfunction, right ventricular/interatrial septum/valve thickness, left ventricular sparkling, or apical sparing. Cardiac hypertrophy and persistent elevation in cardiac troponin in elderly patients are also suggestive of ATTR‐CM. 99mTc‐labelled bone radiotracer scintigraphy is also recommended in patients with characteristic cardiac magnetic resonance findings (e.g. diffuse subendocardial late gadolinium enhancement patterns, native T1 increase, and increase in extracellular volume) or patients with cardiac hypertrophy and bilateral carpal tunnel syndrome.

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