Author + information
- Received June 13, 2019
- Revision received February 11, 2020
- Accepted February 11, 2020
- Published online June 1, 2020.
- Sara Rosengren, MDa,∗∗ (, )
- Tor Skibsted Clemmensen, MD, PhDb,∗,
- Lars Tolbod, PhDc,
- Sven-Olof Granstam, MD, PhDd,
- Hans Eiskjær, MD, PhDb,
- Gerhard Wikström, MD, PhDe,
- Ola Vedin, MD, PhDe,
- Tanja Kero, MD, PhDf,
- Mark Lubberink, PhDf,g,
- Hendrik J. Harms, PhDc,
- Frank A. Flachskampf, MD, PhDd,
- Tomasz Baron, MD, PhDd,
- Kristina Carlson, MD, PhDa,
- Fabian Mikkelsen, MDb,
- Gunnar Antoni, PhDh,
- Niels Frost Andersen, MD, PhDi,
- Steen Hvitfeldt Poulsen, MD, PhDb and
- Jens Sörensen, MD, PhDc,f
- aDepartment of Medical Sciences, Hematology, Uppsala University, Uppsala, Sweden
- bDepartment of Cardiology, Aarhus University Hospital, Aarhus, Denmark
- cDepartment of Nuclear Medicine and PET, Aarhus University Hospital, Aarhus, Denmark
- dDepartment of Medical Sciences, Clinical Physiology, Uppsala University, Uppsala, Sweden
- eDepartment of Medical Sciences, Cardiology, Uppsala University, Uppsala, Sweden
- fDepartment of Surgical Sciences, Radiology, Uppsala University, Uppsala, Sweden
- gDepartment of Medical Physics, Uppsala University, Uppsala, Sweden
- hDepartment of Medicinal Chemistry, Uppsala University, Uppsala, Sweden
- iDepartment of Hematology, Aarhus University Hospital, Aarhus, Denmark
- ↵∗Address for correspondence:
Dr Sara Rosengren, Department of Hematology, Ing 100, pl 2 Akademiska sjukhuset, 751 85 Uppsala, Sweden.
Objectives This dual-site study evaluated the diagnostic accuracy of the method.
Background Pittsburgh compound ([11C]PIB) positron emission tomography (PIB-PET) has shown promise as a specific and noninvasive method for the diagnosis of cardiac amyloidosis (CA).
Methods The study had 2 parts. In the initial study, 51 subjects were included, 36 patients with known CA and increased wall thickness (15 immunoglobulin light chain [AL] and 21 transthyretin [ATTR] amyloidosis) and 15 control patients (7 were nonamyloid hypertrophic and 8 healthy volunteers). Subjects underwent PIB-PET and echocardiography. Sensitivity and specificity of PIB-PET were established for 2 simple semiquantitative approaches, standardized uptake value ratio (SUVR) and retention index (RI). The second part of the study included 11 amyloidosis patients (5 AL and 6 hereditary ATTR) without increased wall thickness to which the optimal cutoff values of SUVR (>1.09) and RI (>0.037 min-1) were applied prospectively.
Results The diagnostic accuracy of visual inspection of [11C]PIB uptake was 100% in discriminating CA patients with increased wall thickness from controls. Semiquantitative [11C]PIB uptake discriminated CA from controls with a 94% (95% confidence interval [CI]: 80% to 99%) sensitivity for both SUVR and RI and specificity of 93% (95% CI: 66% to 100%) for SUVR and 100% (95% CI: 75% to 100%) for RI. [11C]PIB uptake was significantly higher in AL-CA than in ATTR-CA patients (p < 0.001) and discriminated AL-CA from controls with 100% (95% CI: 88% to 100%) accuracy for both the semiquantitative measures. In the prospective group without increased wall thickness, RI was elevated compared to controls (p = 0.001) and 5 of 11 subjects were evaluated as [11C]PIB PET positive.
Conclusions In a dual-center setting, [11C]PIB PET was highly accurate in detecting cardiac involvement in the main amyloid subtypes, with 100% accuracy in AL amyloidosis. A proportion of amyloidosis patients without known cardiac involvement were [11C]PIB PET positive, indicating that the method may detect early stages of CA.
↵∗ Drs. Rosengren and Skibsted Clemmensen contributed equally to this work.
This study received funding from the Swedish Heart-Lung Foundation, Stockholm, Sweden (registration number 20160746). All authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors' institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Cardiovascular Imaging author instructions page.
- Received June 13, 2019.
- Revision received February 11, 2020.
- Accepted February 11, 2020.
- 2020 American College of Cardiology Foundation
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