Björn Pilebro1, Sandra Arvidsson2, Per Lindqvist2, Torbjörn Sundström3, Per Westermark4, Gunnar Antoni5, Ole Suhr6, Jens Sörensen7. 1. Heart Centre, Cardiology, Department of Public Health and Clinical Medicine, Umeå University, 901 85, Umeå, Sweden. bjorn.pilebro@umu.se. 2. Heart Centre, Department of Surgical and Perioperative Sciences, Clinical Physiology, Umeå University, Umeå, Sweden. 3. Department of Radiation Sciences, Diagnostic Radiology, Umeå University, Umeå, Sweden. 4. Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden. 5. Department of Medicinal Chemistry, Uppsala University, Uppsala, Sweden. 6. Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden. 7. Department of Surgical Sciences, Nuclear Medicine & PET, Uppsala University, Uppsala, Sweden.
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. 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. CONCLUSION: 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.
BACKGROUND:DPD scintigraphy has been advocated for imaging cardiac amyloid in ATTRamyloidosis. 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 ATTRamyloidosis. 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 ATTRamyloidosis. METHODS: Ten patients with biopsy-proven V30MATTRamyloidosis 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. CONCLUSION: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 ATTRamyloidosis.
Authors: Dorota M Rowczenio; Islam Noor; Julian D Gillmore; Helen J Lachmann; Carol Whelan; Philip N Hawkins; Laura Obici; Per Westermark; Gilles Grateau; Ashutosh D Wechalekar Journal: Hum Mutat Date: 2014-08-04 Impact factor: 4.878
Authors: Victoria Delgado; Sjoerd A Mollema; Claudia Ypenburg; Laurens F Tops; Ernst E van der Wall; Martin J Schalij; Jeroen J Bax Journal: J Am Soc Echocardiogr Date: 2008-11 Impact factor: 5.251
Authors: Mi-Ae Park; Robert F Padera; Anthony Belanger; Shipra Dubey; David H Hwang; Vikas Veeranna; Rodney H Falk; Marcelo F Di Carli; Sharmila Dorbala Journal: Circ Cardiovasc Imaging Date: 2015-08 Impact factor: 7.792
Authors: Agneta Nordberg; Stephen F Carter; Juha Rinne; Alexander Drzezga; David J Brooks; Rik Vandenberghe; Daniela Perani; Anton Forsberg; Bengt Långström; Noora Scheinin; Mira Karrasch; Kjell Någren; Timo Grimmer; Isabelle Miederer; Paul Edison; Aren Okello; Koen Van Laere; Natalie Nelissen; Mathieu Vandenbulcke; Valentina Garibotto; Ove Almkvist; Elke Kalbe; Rainer Hinz; Karl Herholz Journal: Eur J Nucl Med Mol Imaging Date: 2012-09-08 Impact factor: 9.236
Authors: Agnese Irkle; Alex T Vesey; David Y Lewis; Jeremy N Skepper; Joseph L E Bird; Marc R Dweck; Francis R Joshi; Ferdia A Gallagher; Elizabeth A Warburton; Martin R Bennett; Kevin M Brindle; David E Newby; James H Rudd; Anthony P Davenport Journal: Nat Commun Date: 2015-07-07 Impact factor: 14.919
Authors: Sharmila Dorbala; Yukio Ando; Sabahat Bokhari; Angela Dispenzieri; Rodney H Falk; Victor A Ferrari; Marianna Fontana; Olivier Gheysens; Julian D Gillmore; Andor W J M Glaudemans; Mazen A Hanna; Bouke P C Hazenberg; Arnt V Kristen; Raymond Y Kwong; Mathew S Maurer; Giampaolo Merlini; Edward J Miller; James C Moon; Venkatesh L Murthy; C Cristina Quarta; Claudio Rapezzi; Frederick L Ruberg; Sanjiv J Shah; Riemer H J A Slart; Hein J Verberne; Jamieson M Bourque Journal: J Nucl Cardiol Date: 2019-12 Impact factor: 5.952
Authors: Giorgio Treglia; Andor W J M Glaudemans; Francesco Bertagna; Bouke P C Hazenberg; Paola A Erba; Raffaele Giubbini; Luca Ceriani; John O Prior; Luca Giovanella; Riemer H J A Slart Journal: Eur J Nucl Med Mol Imaging Date: 2018-04-23 Impact factor: 9.236
Authors: Peter P Rainer; Peihong Dong; Matteo Sorge; Justyna Fert-Bober; Ronald J Holewinski; Yuchuan Wang; Catherine A Foss; Steven S An; Alessandra Baracca; Giancarlo Solaini; Charles G Glabe; Martin G Pomper; Jennifer E Van Eyk; Gordon F Tomaselli; Nazareno Paolocci; Giulio Agnetti Journal: Circ Res Date: 2018-02-26 Impact factor: 17.367