title: Usefulness of (99m)Tc-HMDP scintigraphy for the etiologic diagnosis and prognosis of cardiac amyloidosis.
abstract: BACKGROUND
  Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage. The three main types are monoclonal light chain (AL), wild-type transthyretin (wt-TTR) and mutated transthyretin (m-TTR) amyloidosis. Cardiac amyloidosis (CA) raises diagnostic challenges.
  
  
  OBJECTIVE
  To assess the diagnostic accuracy of (99m)Tc-HMDP-scintigraphy for typing CA, differentiating CA from non-amyloid left ventricle hypertrophy (LVH), and predicting outcomes.
  
  
  METHODS
  121 patients with suspected CA underwent (99m)Tc-HMDP-scintigraphy in addition to standard investigations.
  
  
  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. (99m)Tc-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.
  
  
  CONCLUSIONS
  This preliminary study suggests that (99m)Tc-HMDP-scintigraphy may aid differentiation between transthyretin and AL-CA as well as CA from other LVHs. (99m)Tc-HMDP-scintigraphy appears to provide prognostic information in CA.
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|d INSERM U955, GRC Réseau Amylose Mondor , Créteil , France .|e DHU ATVB , Créteil , France .
authors.forename: Arnault
authors.initials: A
authors.lastname: Galat
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|e DHU ATVB , Créteil , France .|f Department of Nuclear Medicine , AP-HP, Henri-Mondor Teaching Hospital , Créteil , France .
authors.forename: Jean
authors.initials: J
authors.lastname: Rosso
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|d INSERM U955, GRC Réseau Amylose Mondor , Créteil , France .|e DHU ATVB , Créteil , France .|g INSERM Clinical Investigation Centre 006 , Créteil , France , and.
authors.forename: Aziz
authors.initials: A
authors.lastname: Guellich
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|e DHU ATVB , Créteil , France .|f Department of Nuclear Medicine , AP-HP, Henri-Mondor Teaching Hospital , Créteil , France .
authors.forename: Axel
authors.initials: A
authors.lastname: Van Der Gucht
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|e DHU ATVB , Créteil , France .|g INSERM Clinical Investigation Centre 006 , Créteil , France , and.
authors.forename: Stephane
authors.initials: S
authors.lastname: Rappeneau
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|e DHU ATVB , Créteil , France .
authors.forename: Diane
authors.initials: D
authors.lastname: Bodez
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|e DHU ATVB , Créteil , France .
authors.forename: Soulef
authors.initials: S
authors.lastname: Guendouz
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|e DHU ATVB , Créteil , France .
authors.forename: Claire-Marie
authors.initials: CM
authors.lastname: Tissot
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|e DHU ATVB , Créteil , France .
authors.forename: Luc
authors.initials: L
authors.lastname: Hittinger
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|e DHU ATVB , Créteil , France .
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Dubois-Randé
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|e DHU ATVB , Créteil , France .|h Department of Neurology , AP-HP, Henri-Mondor Teaching Hospital , Créteil , France.
authors.forename: Violaine
authors.initials: V
authors.lastname: Plante-Bordeneuve
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|e DHU ATVB , Créteil , France .|f Department of Nuclear Medicine , AP-HP, Henri-Mondor Teaching Hospital , Créteil , France .
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Itti
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|e DHU ATVB , Créteil , France .|f Department of Nuclear Medicine , AP-HP, Henri-Mondor Teaching Hospital , Créteil , France .
authors.forename: Michel
authors.initials: M
authors.lastname: Meignan
authors.affiliation: a UPEC , Créteil , France .|b Mondor Amyloidosis Network , Créteil , France .|c Department of Cardiology, AP-HP , Henri-Mondor Teaching Hospital , Créteil , France .|d INSERM U955, GRC Réseau Amylose Mondor , Créteil , France .|e DHU ATVB , Créteil , France .|g INSERM Clinical Investigation Centre 006 , Créteil , France , and.
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000473:pathology; D004452:Echocardiography; D005260:Female; D006331:Heart Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals / Q000008:administration & dosage*; D013669:Technetium Tc 99m Medronate / Q000008:administration & dosage / Q000031:analogs & derivatives*
chemical_list: D019275:Radiopharmaceuticals; C024226:technetium Tc 99m hydroxymethylene diphosphonate; D013669:Technetium Tc 99m Medronate
keywords: Bone tracer; diphosphonate; extracellular matrix; light chain immunoglobulin; nuclear medicine; protein deposition; transthyretin