technetium-tc-99m-pyrophosphate and Polyneuropathies

To determine whether technetium-99m-pyrophosphate (Tc-99m-PYP) scanning or two-dimensional echocardiography can detect amyloid heart disease in an earlier stage of familial amyloid 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 images (p less than 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 familial amyloid polyneuropathy than two-dimensional echocardiography.

Topics: Adult; Aged; Amyloidosis; Cardiomyopathies; Diphosphates; Echocardiography; Female; Humans; Male; Middle Aged; Polyneuropathies; Radionuclide Imaging; Technetium; Technetium Tc 99m Pyrophosphate

A patient with familial amyloid polyneuropathy and congestive heart failure underwent myocardial imaging using technetium-99m pyrophosphate. Planar scintigraphy showed an intense, diffuse biventricular uptake of the radiotracer. Single-photon emission computed tomography demonstrated an unevenly distributed uptake of the isotope. The greatest activity corresponded to regions with marked echocardiographic changes. Emission tomography may aid in assessing the degree and distribution of the infiltrative lesions in cardiac amyloidosis.

Topics: Aged; Amyloidosis; Diphosphates; Echocardiography; Heart; Humans; Male; Polyneuropathies; Technetium; Technetium Tc 99m Pyrophosphate; Tomography, Emission-Computed

Topics: Adult; Aged; Amyloidosis; Cardiomyopathies; Diphosphates; Female; Gallium Radioisotopes; Humans; Male; Middle Aged; Polyneuropathies; Radionuclide Imaging; Technetium; Technetium Tc 99m Medronate; Technetium Tc 99m Pyrophosphate

Topics: Amyloidosis; Diphosphates; Heart; Humans; Male; Middle Aged; Myocardium; Polyneuropathies; Radionuclide Imaging; Technetium; Technetium Tc 99m Pyrophosphate

To assess the clinical significance of technetium-99m-pyrophosphate (Tc-99m-PYP), -methylene diphosphonate (Tc-99m-MDP) and thallium-201 (Tl-201) myocardial scintigraphy in the diagnosis of cardiac amyloidosis and in the differential diagnosis of cardiac diseases, 12 patients with biopsy-proved systemic amyloidosis (seven with familial amyloid polyneuropathy (FAP) and five with primary amyloidosis) were investigated. The results obtained were as follows: In 10 patients (six with FAP and four with primary amyloidosis) studied by Tc-99m-PYP scintigraphy, two (FAP one, primary amyloidosis one) had diffusely positive myocardial uptake, which was of greater intensity than that of the sternum. Six (four FAP; two primary amyloidosis) also had diffusely positive myocardial uptakes, but the intensity was less than that of the sternum. The remaining two (one FAP; one primary amyloidosis) had only equivocal myocardial uptakes. Two of these patients also had hepatic uptakes and another had both hepatic and thyroid uptakes. The intensity of myocardial uptake of Tc-99m-PYP in patients with echocardiographic left ventricular hypertrophy and/or highly refractile myocardial echoes, so-called granular sparkling appearance (GS) was slightly greater than that in patients with neither myocardial hypertrophy nor GS. FAP had slightly less intensity than primary amyloidosis. In 29 persons with other cardiac diseases and normal subjects examined by Tc-99m-PYP scintigraphy, seven (two dilated cardiomyopathy; two sarcoidosis; three hypertensive heart disease) also had diffusely positive myocardial scans of mild or moderate degree. However, none of them had marked myocardial or other tissue uptakes. Both Tc-99m-PYP and -MDP scintigraphic studies were performed in four patients (three FAP; one primary amyloidosis). In Tc-99m-MDP scintigraphy, diffusely positive myocardial uptakes were observed in two patients with FAP and the remaining two had negative scans. The intensity of Tc-99m-MDP myocardial uptake in each patient was significantly lower than that of Tc-99m-PYP uptake. Tl-201 scintigraphy was carried out in 10 patients (six FAP; four primary amyloidosis). Left ventricular hypertrophy was found in six patients and right ventricular visualization in five. Although electrocardiograms in seven of 10 patients showed QS patterns in the right to mid precordial leads, similar to that seen in antero-septal and extensive anterior myocardial infarctions, neither myocardial perfusion defec

Topics: Aged; Amyloidosis; Biopsy; Diagnosis, Differential; Diphosphates; Diphosphonates; Echocardiography; Electrocardiography; Endocardium; Female; Heart; Humans; Male; Middle Aged; Myocardial Infarction; Polyneuropathies; Radioisotopes; Radionuclide Imaging; Technetium; Technetium Compounds; Technetium Tc 99m Medronate; Technetium Tc 99m Pyrophosphate; Thallium