Early Detection of Amyloid Heart Disease by Technetium-99M-Pyrophosphate Single-Photon Emission-Computed Tomography : A Study with Familial Amyloid Polyneuropathy
Eighteen patients with familial amyloid polyneuropathy (FAP) were studied to determine whether technetium-99m-pyrophosphate (Tc-99m-PYP) scintigraphy or two-dimensional echocardiography (2-DE) can detect myocardial involvement in an earlier stage. Thirteen of them were considered to be in the early stage of myocardial amyloid infiltrative process because they showed no clinical evidence of overt heart disease, as well as normal ventricular wall thickness and normal values for left ventricular systolic function. Although planner scintigraphy showed mild or moderate myocardial uptake in six (46 %) of the 13 patients, single-photon emission-computed tomography, performed in 12 of them, demonstrated an unevenly distributed biventricular uptake in ten (83 %), including four who were judged to be negative by the planner scanning. On the other hand, none had characteristic highly refractile myocardial echoes on the 2-DE images. In additional 95 control patients with nonischemic heart disease, planner scintigraphy showed diffuse positive pyrophosphate scans of the heart in five (4%) of them and 2-DE demonstrated granular sparkling echoes in 10 (10%). In conclusion, (1) planner Tc-99m-PYP scanning is a more sensitive and specific procedure to detect amyloid heart disease in the earlier stage of FAP than 2-DE and (2) single-photon emission-computed tomography may be more sensitive for diagnosing this condition.
KeywordsLeft Ventricular Systolic Function Aortic Valve Stenosis Fractional Shorten Cardiac Amyloidosis Myocardial Uptake
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