Abstract
Background Familial amyloid polyneuropathy (FAP) is a severe hereditary disease, due to production by the liver of a genetic variant transthyretin (TTR) resulting in tissue amyloid deposits. Cardiac involvement is of major prognostic value. Diphosphonate scintigraphy has been proposed as a diagnostic tool for TTR-related cardiac amyloidosis, but there is no consensus on the optimal radiopharmaceutical. Consequently, we compared the cardiac uptake of two 99mTc-labelled tracers: diphosphono-propanedicarboxylic acid (DPD) and hydroxymethylene diphosphonate (HMDP) in patients with TTR-FAP.
Highlights
Familial amyloid polyneuropathy (FAP) is a severe hereditary disease, due to production by the liver of a genetic variant transthyretin (TTR) resulting in tissue amyloid deposits
Quantification of soft tissues uptake was performed by use of ratio between average counts of a region of interest (ROI) drawn over the lumbar spine and a ROI drawn over soft tissues of the lower limb (B/ST) on the WB acquisition
Quantitative parameters derived from whole body acquisition were significantly greater with diphosphono-propanedicarboxylic acid (DPD) compared to hydroxymethylene diphosphonate (HMDP) (H/WBtotal: 3.8±2.7 vs. 2.4±2.1 respectively; p=0.002 and H/WBaverage: 5.2±2.2 vs. 4.3±1.3 respectively; p=0.01) as well as H/L derived from SPECT acquisition (3.9±3.7 vs. 2.0±1.9 respectively; p=0.001)
Summary
Familial amyloid polyneuropathy (FAP) is a severe hereditary disease, due to production by the liver of a genetic variant transthyretin (TTR) resulting in tissue amyloid deposits. Cardiac involvement is of major prognostic value. Diphosphonate scintigraphy has been proposed as a diagnostic tool for TTR-related cardiac amyloidosis, but there is no consensus on the optimal radiopharmaceutical. We compared the cardiac uptake of two 99mTc-labelled tracers: diphosphono-propanedicarboxylic acid (DPD) and hydroxymethylene diphosphonate (HMDP) in patients with TTR-FAP
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call