Abstract
Background DPD scintigraphy has been advocated for imaging cardiac amyloid in ATTR amyloidosis. PET utilizing 11C-Pittsburgh compound B (PIB) is the gold standard for imaging brain amyloid in Alzheimer’s disease. PIB was recently shown to identify cardiac amyloidosis in both AL and ATTR amyloidosis. In the ATTR population, two types of amyloid fibrils exist, one containing fragmented and full-length TTR (type A) and the other only full-length TTR (type B). The aim of this study was to further evaluate PIB-PET in patients with hereditary ATTR amyloidosis.
Highlights
Severe cardiac involvement in hereditary V30M transthyretin (TTR) amyloidosis (ATTRV30M) has been linked to amyloid containing fragmented TTR, fibril type A and “early onset” mainly neurological disease to fibrils containing only full length TTR, type B
A number of different modalities to diagnose for cardiac imaging amyloid deposition in the heart have been developed, but so far, none have been proven to identify cardiac amyloid deposits in ATTR
All patients had pathological 11C-Pittsburgh compound B (PIB) uptake but the pattern of uptake differed between the two groups
Summary
Positron Emission Tomography (PET) utilizing Pittsburgh compound B (PIB) detects amyloid heart deposits in hereditary transthyretin amyloidosis (ATTR). Björn Pilebro1*, Per Lindqvist, Sandra Gustafsson, Per Westermark, Torbjörn Sundström, Gunnar Antoni, Ole Suhr, Jens Sörensen. From First European Congress on Hereditary ATTR amyloidosis Paris, France. From First European Congress on Hereditary ATTR amyloidosis Paris, France. 2-3 November 2015
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