Abstract
Results CA was diagnosed in all AL (n=14) and wt-TTR (n=21). Among m-TTR (n=34), 26 had CA, 4 neuropathy without CA and 4 were asymptomatic carriers. Of the 52 patients with non-amyloid heart disease, 37 had LVH and served as controls. 99mTc-HMDP cardiac uptake occurred in all wt-TTR, in m-TTR with CA except two, and in one AL. A visual score ≥2 was 100% specific for diagnosing TTR-CA. Among TTR-CA, heart-to-skull retention (HR/SR) correlated with CA severity (LVEF and NT-proBNP). Median follow-up was 111 days (50;343). In a multivariate Cox model including clinical, echocardiographic, and scintigraphic variables, NYHA III-IV and HR/SR>1.94 predicted acute heart failure and/or death.
Highlights
Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage
Cardiac amyloidosis (CA) was diagnosed in all are monoclonal light chain (AL) (n=14) and wild-type transthyretin (wt-TTR) (n=21)
Of the 52 patients with non-amyloid heart disease, 37 had left ventricle hypertrophy (LVH) and served as controls. 99mTc-HMDP cardiac uptake occurred in all wt-TTR, in mutated transthyretin (m-TTR) with CA except two, and in one AL
Summary
Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage. Usefulness of 99mTc-HMDP scintigraphy for the etiologic diagnosis and prognosis of cardiac amyloidosis Arnault Galat*, Jean Rosso, Aziz Guellich, Axel Van Der Gucht, Jean-Luc Dubois-Randé, Violaine Plante-Bordeneuve, Emmanuel Itti, Thibaud Damy From First European Congress on Hereditary ATTR amyloidosis Paris, France.
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