Early identification of amyloid heart disease by technetium-99m-pyrophosphate scintigraphy: A study with familial amyloid polyneuropathy

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Abstract

To determine whether technetium-99m-pyrophosphate (Tc-99m-PYP) scanning or two-dimensional echocardiography can detect amyloid heart disease in an earlier stage of famillial amylold polyneuropathy, 15 patients were examined. Although 10 of the 15 patients had no clinical evidence of congestive heart failure, as well as normal ventricular wall thickness and normal values for left ventricular systolic function, five (50%) of them showed mild or moderate myocardial uptake. On the other hand, none had characteristic highly refractile myocardial echoes on the two-dimensional echocardiographic lmages (p < 0.01), and values for diastolic function were reduced in four of the five and normal in the remaining one. In 85 control subjects, diffuse positive pyrophosphate scans of the heart were found in four (5%) of them (three with dilated cardiomyopathy and one with sarcoidosis), and highly refractile granular sparkling echoes were observed in nine (11%) (five with hypertrophic cardiomyopathy, three with aortic stenosis, and one with hypereosinophilic syndrome). We conclude that Tc-99m-PYP scanning is a more sensitive and specific method and may have the potential ability to detect amyloid heart disease in the earlier stage of famllial amyloid polyneuropathy than two-dimensional echocardiography.

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Cited by (45)

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  • Prevalence of wild type ATTR assessed as myocardial uptake in bone scan in the elderly population

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    In addition, a large proportion of individuals was receiving diuretics and had elevated concentrations of natriuretic peptides. These results support the view that TTR deposition is a continuous process and that the cardiac uptake of bone tracers precedes clinical manifestations of HF, allowing the early diagnosis of TTR cardiac amyloidosis even before the appearance of other echocardiographic and electrocardiographic abnormalities or overt clinical manifestations [10, 18–20]. The present study has some limitations, mainly derived from its retrospective nature.

  • Lung uptake during 99mTc-hydroxymethylene diphosphonate scintigraphy in patient with TTR cardiac amyloidosis: An underestimated phenomenon

    2018, International Journal of Cardiology
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    Amyloidoses are rare diseases characterized by extracellular deposition of protein-derived fibrils which show apple-green birefringence when stained with Congo red and viewed under polarized light in various tissues and organs, including the heart [1]. Full body scintigraphy using bone tracers plays an important role in defining the type of amyloidosis and in diagnosing the heart involvement (cardiac amyloidosis, CA) [2–10]. Pathologically, involvement in the amyloid deposition of the respiratory tract is common [11–14] and may involve the lungs in 4 distinct forms: lymphatic, diffuse alveolar septal, nodular parenchymal, and tracheobronchial, with the former two patterns commonly present in patients with systemic involvement and the latter two occurring more commonly in localized forms.

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    Despite few cases reporting soft-tissue 99mTc- methylene-diphosphonate (MDP) uptake in systemic amyloidosis [36] and myocardial 99mTc-MDP uptake in wt-TTR-related amyloidosis [37,38], and despite one study suggesting better contrast than 99mTc-PYP [26] possibly explained by different biological characteristics [39], this tracer was quickly abandoned because of its lower diagnostic performance in demonstrating amyloid deposits within the heart [33,40]. 99mTc-PYP [17,19,24–30,41] and 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) [31–33,42–44] are nowadays the most widely bone tracer studied for TTR-related CA imaging. However, few case had reported hydroxymethylene-diphosphonate (HMDP) uptake in CA [45] and a recent multicenter study over 1200 patients has shown that HMDP can be used with the same proprieties than others tracers [34].

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Supported in part by grants from the Ministry of Health and Welfare Primary Amloidosis Research Committee, Japan.

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