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| Name | Class |
|---|---|
| Pfizer | INDUSTRY |
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Introduction: Transthyretin cardiac amyloidosis (ATTR) is an important cause of heart failure. Cardiac planar radionuclide imaging using 99mTc-labeled bone seeking radiopharmaceuticals is used as a noninvasive diagnostic criterion in patients without detectable monoclonal protein. The visual assessment remains the main noninvasive criterion for the diagnosis. Medical therapy using tafamidis meglumine that binds to transthyretin and prevents amyloidogenesis, recently demonstrated a reduction in all-cause mortality and cardiovascular-related hospitalizations. As a consequence, there is a need for quantitative approaches that would be useful for diagnosis and prognosis assessment but also for the evaluation of patient therapeutic response.
Materials and methods: The investigators aim to include 35 patients with a suspected diagnosis of cardiac ATTR amyloidosis in whom a cardiac planar radionuclide imaging using 99mTc-labeled bone seeking radiopharmaceuticals is planned as part of routine noninvasive diagnosis work-up. Using a test-retest approach, the aim is to compare a quantitative method vs. conventional semi-quantitative approaches for the assessment of cardiac uptake of bone radiopharmaceuticals using new 3D CZT-based SPECT-CT cameras in patients with suspected cardiac ATTR amyloidosis. The investigators estimated that 20 patients will have a diagnosis of cardiac ATTR amyloidosis. In the latter patients, the aim is to evaluate the impact of 6-month therapy using tafamidis on quantitative and semi-quantitative assessment of cardiac uptake of bone radiopharmaceuticals Perspectives: This new non invasive imaging techniques for the quantitative assessment of the amyloid burden in patients with cardiac ATTR amyloidosis may help identify the responders and the patients who should benefit from dose intensification.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| No cardiac ATTR amyloidosis | Other | Cardiac echocardiography at baseline and whole-body & CZT bone tracer imaging (SPECT). day 3 - day 10 : second whole-body & CZT bone tracer imaging (SPECT), test-retest Bone tracer imaging Perugini 0 : no cardiac TTR amyloidosis No further follow-up. |
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| Cardiac ATTR amyloidosis, no treatment with tafamidis planned | Other | Cardiac echocardiography at baseline and whole-body & CZT bone tracer imaging (SPECT). day 3 - day 10 : second whole-body & CZT bone tracer imaging (SPECT), test-retest Bone tracer imaging demonstrating cardiac ATTR amyloidosis but no treament with tafamidis planned. Cardiac echocardiography at 3 months and whole-body & CZT bone tracer SPECT and cardiac echocardiography at 6 months |
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| Cardiac ATTR amyloidosis, treatment with tafamidis planned | Other | Cardiac echocardiography at baseline and whole-body & CZT bone tracer imaging (SPECT). day 3 - day 10 : second whole-body & CZT bone tracer imaging (SPECT), test-retest Bone tracer imaging demonstrating cardiac ATTR amyloidosis. Start of the treament with tafamidis. Cardiac echocardiography at 3 months and whole-body & CZT bone tracer SPECT and cardiac echocardiography at 6 months |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Whole-body & CZT bone tracer imaging (SPECT) | Diagnostic Test | Quantitative analysis of myocardial uptake of 99mTc-labeled bone radiopharmaceuticals using new whole-body CZT-based SPECT-CT cameras |
| Measure | Description | Time Frame |
|---|---|---|
| Test-retest reproducibility | Test-retest repeatability will be assessed by comparing the results from two successive 3D CZT imaging examinations performed in two distinct baseline imaging sessions. Repeatability will be evaluated by the mean absolute difference and the coefficient of variation (COV) (mean relative difference) between the results of the assessment of the 3D-myocardial uptake of bone tracers in the two examinations. A measurement will be considered reproducible when COV will be below 20%. Furthermore, the repeatability will also be assessed using the interclass correlation coefficient (ICC) and its 95% confidence interval (95%CI) under an ANOVA random effect model, and Bland Altman plots. | 10 days |
| Correlations between the quantitative assessment of the myocardial uptake of bone tracers and semi-quantitative approaches | Correlations between the quantitative assessment of the myocardial uptake (COV) of bone tracers and semi-quantitative approaches will be performed by using linear regression analysis performed by the least squares method and Pearson's correlation coefficient r and Bland Altman plots. | 10 days |
| Comparison between the quantitative assessment of the myocardial uptake of bone tracers (COV) between baseline and follow-up imaging of each patient, 6 months after the beginning of the treatment by tafamidis | The investigators will compare baseline and follow-up imaging of each patient, 6 months after the beginning of the treatment by tafamidis using paired comparison for quantitative data and concordance tests (Kappa test) for semi-quantitative data. | 6 months |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Damien Legallois, MD | Contact | 33230165709 | damien.legallois@unicaen.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU de Caen | Recruiting | Caen | 14000 | France | ||
| Clinique du Bois |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40518108 | Derived | Legallois D, Turcan L, Agostini D, Manrique A. Test-retest reproducibility of myocardial 99mTc-HMDP uptake quantification by SPECT-CT in suspected transthyretin cardiac amyloidosis. J Nucl Cardiol. 2025 Sep;51:102273. doi: 10.1016/j.nuclcard.2025.102273. Epub 2025 Jun 13. |
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| ID | Term |
|---|---|
| D028227 | Amyloid Neuropathies, Familial |
| ID | Term |
|---|---|
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D009422 | Nervous System Diseases |
| D017772 | Amyloid Neuropathies |
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| ID | Term |
|---|---|
| D015899 | Tomography, Emission-Computed, Single-Photon |
| ID | Term |
|---|---|
| D014055 | Tomography, Emission-Computed |
| D007090 | Image Interpretation, Computer-Assisted |
| D003952 | Diagnostic Imaging |
| D019937 | Diagnostic Techniques and Procedures |
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| Recruiting |
| Lille |
| 59000 |
| France |
| D010523 | Peripheral Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D028226 | Amyloidosis, Familial |
| D008661 | Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D000686 | Amyloidosis |
| D057165 | Proteostasis Deficiencies |
| D003933 | Diagnosis |
| D007089 | Image Enhancement |
| D010781 | Photography |
| D011877 | Radionuclide Imaging |
| D014054 | Tomography |
| D003947 | Diagnostic Techniques, Radioisotope |