title: Positron emission tomography (PET) utilizing Pittsburgh compound B (PIB) for detection of amyloid heart deposits in hereditary transthyretin amyloidosis (ATTR).
abstract: BACKGROUND
  DPD scintigraphy has been advocated for imaging cardiac amyloid in ATTR amyloidosis. PET utilizing11C-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.
  
  
  METHODS
  Ten patients with biopsy-proven V30M ATTR amyloidosis and discrete or no signs of cardiac involvement were included. Patients were grouped according to TTR-fragmentation. All underwent DPD scintigraphy, echocardiography, and PIB-PET. A left ventricular PIB-retention index (PIB-RI) was established and compared to five normal volunteers.
  
  
  RESULTS
  PIB-RI was increased in all patients (P < 0.001), but was significantly higher in type B than in type A (0.129 ± 0.041 vs 0.040 ± 0.006 min-1, P = 0.009). Cardiac DPD uptake was elevated in group A and absent in group B.
  
  
  CONCLUSIONS
  PIB-PET, in contrast to DPD scintigraphy, has the potential to specifically identify cardiac amyloid depositions irrespective of amyloid fibril composition. The heart appears to be a target organ for amyloid deposition in ATTR amyloidosis.
authors.affiliation: Heart Centre, Cardiology, Department of Public Health and Clinical Medicine, Umeå University, 901 85, Umeå, Sweden. bjorn.pilebro@umu.se.
authors.forename: Björn
authors.initials: B
authors.lastname: Pilebro
authors.affiliation: Heart Centre, Department of Surgical and Perioperative Sciences, Clinical Physiology, Umeå University, Umeå, Sweden.
authors.forename: Sandra
authors.initials: S
authors.lastname: Arvidsson
authors.affiliation: Heart Centre, Department of Surgical and Perioperative Sciences, Clinical Physiology, Umeå University, Umeå, Sweden.
authors.forename: Per
authors.initials: P
authors.lastname: Lindqvist
authors.affiliation: Department of Radiation Sciences, Diagnostic Radiology, Umeå University, Umeå, Sweden.
authors.forename: Torbjörn
authors.initials: T
authors.lastname: Sundström
authors.affiliation: Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden.
authors.forename: Per
authors.initials: P
authors.lastname: Westermark
authors.affiliation: Department of Medicinal Chemistry, Uppsala University, Uppsala, Sweden.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Antoni
authors.affiliation: Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
authors.forename: Ole
authors.initials: O
authors.lastname: Suhr
authors.affiliation: Department of Surgical Sciences, Nuclear Medicine & PET, Uppsala University, Uppsala, Sweden.
authors.forename: Jens
authors.initials: J
authors.lastname: Sörensen
mesh_terms: D000368:Aged; D000544:Alzheimer Disease / Q000000981:diagnostic imaging; D000682:Amyloid / Q000032:analysis*; D028227:Amyloid Neuropathies, Familial / Q000000981:diagnostic imaging*; D000814:Aniline Compounds; D001706:Biopsy; D001921:Brain / Q000000981:diagnostic imaging; D002250:Carbon Radioisotopes; D004452:Echocardiography; D005260:Female; D064368:Healthy Volunteers; D006321:Heart / Q000000981:diagnostic imaging*; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D008875:Middle Aged; D000072078:Positron Emission Tomography Computed Tomography; D049268:Positron-Emission Tomography; D011877:Radionuclide Imaging; D013844:Thiazoles
chemical_list: C475519:2-(4'-(methylamino)phenyl)-6-hydroxybenzothiazole; D000682:Amyloid; D000814:Aniline Compounds; D002250:Carbon Radioisotopes; C000615233:Carbon-11; D013844:Thiazoles
keywords: Cardiomyopathy; Pittsburgh compound B; amyloidosis