scholarly journals Quantitation vs visual assessment of 99mTc-DPD cardiac uptake in patients with suspected cardiac amyloidosis

2021 ◽  
Vol 22 (Supplement_3) ◽  
Author(s):  
I Casans-Tormo ◽  
A Canoves-Llombart
Keyword(s):  
Cardiac Death ◽  
Cardiac Amyloidosis ◽  
Quantitative Methods ◽  
Activity Ratio ◽  
Visual Assessment ◽  
Whole Body ◽  
Cardiac Uptake ◽  
Visual Score ◽  
Planar Images

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): Universitary Clinic Hospital of Valencia Aim Cardiac uptake of 99mTc-DPD has proved its diagnostic efficacy in transthyretin cardiac amyloidosis(ATTR). We compared the usual visual assessment with two quantitative methods to evaluate cardiac activity and its possible relation with clinical follow-up. Methods We have studied 37 successive patients(p):32 men, 52-90 y/o (79.6 ± 9.3), submitted by suspected cardiac amyloidosis TTR by echo/cardiac MRI. After IV administration of 21.5 ± 3.4mCi of 99mTc-DPD, we obtained early (at 5min) and late (at 3h) whole body(WB) planar images and late SPECT. We assessed cardiac uptake in planar images by visual score (0-absent, 1-cardiac uptake lower than bone uptake, 2-equal, 3- higher than bone uptake), considering 2-3 score compatible with ATTR in absence of abnormal light chains on serum-urine. We obtained quantitative evaluation by heart/contralateral thoracic activity ratio (H/CL) in late images and from early and late WB images the heart retention(HR) ratio and heart/whole body(HWB) activity ratio. Clinical follow-up (12.4 ± 8.3 months) considering as cardiac events(CE): cardiac death and heart failure(HF) admissions. Results Visual score 0(11p), 1(2p), 2(4p) and 3(20p), considering group1(0-1) not suggestive of ATTR and group2(2-3) compatible with ATTR. SPECT showed biventricular uptake with septal predominance in group2. H/CL index was 2.23 ± 0.54(group2) vs 1.05 ± 0.10(group1) p < 0.001), according to published(≥1.5 compatible with ATTR). HR and HWB ratios were also significantly higher in group2 vs group1, respectively 6 ± 3.14vs2.17 ± 0.4(p < 0.001) and 6.51 ± 1.97vs2.65 ± 0.49(p < 0.001). There were a trend to higher values in p with visual score3 than 2, not reaching statistically significant(only 4p with visual score2). There were significant correlation between H/CL-HR(r:0.66,p < 0.01), H/CL-HWB(r:0.85,p < 0.001) and HR-HWB(r:0.85,p < 0.001). After follow-up we detected 6 CE(3p with HF admission, 3p cardiac death): 5p with visual score3 and one with 2, all 6p with high mean values of H/CL: 2.28 ± 0.76, HR: 6.02 ± 3.4 and HWB:5.97 ± 1.69. Conclusion We have found excellent correlation between visual score and the evaluated methods of quantitation of 99mTc-DPD cardiac uptake in our patients referred by suspected cardiac amyloidosis. These quantitative methods could be a helpful tool to correctly identify some patients with doubtful activity and could be useful in the follow-up evaluation, although it is necessary to study a greater number of patients.

scholarly journals Whole-Body SPECT/CT versus Planar Bone Scan with Targeted SPECT/CT for Metastatic Workup

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Olivier Rager ◽  
René Nkoulou ◽  
Nadia Exquis ◽  
Valentina Garibotto ◽  
Claire Tabouret-Viaud ◽  
...  
Keyword(s):  
Bone Metastases ◽  
Whole Body ◽  
Significant Difference ◽  
Planar Images ◽  
History Of ◽  
Bone Scans ◽  
Base Of The Skull ◽  
History Of Cancer ◽  
Higher Sensitivity

Purpose. The use of SPECT/CT in bone scans has been widespread in recent years, but there are no specific guidelines concerning the optimal acquisition protocol. Two strategies have been proposed: targeted SPECT/CT for equivocal lesions detected on planar images or systematic whole-body SPECT/CT. Our aim was to compare the diagnostic accuracy of the two approaches. Methods. 212 consecutive patients with a history of cancer were referred for bone scans to detect bone metastases. Two experienced readers randomly evaluated for each patient either planar images with one-field SPECT/CT targeted on equivocal focal uptakes (targeted SPECT/CT) or a whole-body (two-field) SPECT/CT acquisition from the base of the skull to the proximal femurs (whole-body SPECT/CT). The exams were categorized as “nonmetastatic,” “equivocal,” or “metastatic” on both protocols. The presence or absence of any extra-axial skeletal lesions was also assessed. The sensitivity and specificity of both strategies were measured using the results of subsequent imaging follow-up as the reference standard. Results. Whole-body SPECT/CT had a significantly higher sensitivity than targeted SPECT/CT to detect bone metastases (p=0.0297) and to detect extra-axial metastases (p=0.0266). There was no significant difference in specificity among the two approaches. Conclusion. Whole-body SPECT/CT is the optimal modality of choice for metastatic workup, including detection of extra-axial lesions, with improved sensitivity and similar specificity compared to targeted SPECT/CT.

scholarly journals 201 Clinical presentation profiles and natural history of cardiac amyloidosis: long-term monocentric analysis (1990–2020)

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Aldostefano Porcari ◽  
Valentina Allegro ◽  
Linda Pagura ◽  
Francesca Longo ◽  
Maddalena Rossi ◽  
...  
Keyword(s):  
Natural History ◽  
Cardiac Death ◽  
Cardiac Amyloidosis ◽  
Outcome Measure ◽  
Favourable Outcome ◽  
Secondary Outcome ◽  
Historical Cohort ◽  
All Cause Mortality ◽  
History Of

Abstract Aims Cardiac Amyloidosis (CA) is considered a rare condition comprising different entities. Epidemiological data are limited and the natural history of disease is largely unknown. Understanding the clinical profiles at presentation, the impact of novel diagnostic strategies and the prognostic predictors at baseline will improve patients’ clinical management. We aimed to examine the epidemiology and natural history of CA in the last 30 years at a tertiary referral centre for amyloidosis. Methods and results Data of patients included in the prospective ‘Cardiac Amyloidosis Registry’ of Trieste from January 1990 to December 2020 were extracted from an electronical database and analysed. The diagnosis of CA was made in presence of (a) amyloid deposition found at endomyocardial biopsy (EMB), or (b) high grade cardiac uptake at bisphosphonate scintigraphy in absence of monoclonal components. Cardiological data of patients included (i) clinical examination, (ii) electrocardiogram (ECG), (iii) echocardiography and (iv) medications. The primary outcome measure was all-cause mortality. The secondary outcome measure was cardiac death. Of the 143 patients with CA included in this analysis, 77 (54%) were diagnosed before 2016 (historical cohort) and 66 (46%) ≥2016 (contemporary cohort). Light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis accounted for 49% and 38%, respectively, of all CA patients. CA of unknown aetiology accounted for 13% of cases. CA was diagnosed by EMB in 98 (69%) patients and by cardiac scintigraphy with bone tracers in 45 (31%) patients. Patients in the contemporary cohort (67% ATTR-CA) were diagnosed more frequently by non-invasive approach compared to those in the historical cohort. At a median global Follow-up of 36 months, a more favourable outcome was found in a) patients from the contemporary cohort compared to those from the historical cohort (P < 0.001), b) ATTR- compared to AL-CA (at 18 months of Follow-up 42 (85%) ATTR patients and 32 (60%) AAL patients were alive, P = 0.013), and, (c) patients diagnosed non-invasively by scintigraphy rather than by histology (at 18 months of Follow-up 36 (80%) of patients diagnosed by cardiac scintigraphy and 57 (60%) of those diagnosed by histology were alive, P = 0.001). Of note, while no difference in outcome was found among AL- and ATTR-CA in the historical cohort, ATTR-CA patients had lower all-cause mortality and cardiac death than AL-CA patients in the contemporary cohort. Overall, death for end stage HF was more prevalent in patients with AL- than ATTR-CA (58% vs. 25%, P = 0.002). At univariable analysis, ACE-i and beta blockers (BBs) therapy were associated with a more favorable outcome [HR: 0.38, (0.26–0.60, P < 0.001) and HR: 0.53 (0.33–0.85, P = 0.008), respectively], while experiencing a previous syncope and having low QRS voltages at surface ECG portended a worse overall survival [HR: 2.42 (1.3–4.5, P = 0.006) and HR: 1.94 (1.3–3.0, P = 0.002), respectively]. At multivariate analysis, treatment with ACE-i, BBs, and syncope had independent prognostic value [HR 0.41 (0.23–0.71, P = 0.002); HR 0.50 (0.31–0.82, P = 0.007), and HR: 2.1 (1.0–4.1, P = 0.040); respectively]. Conclusions The epidemiology and natural history of CA has been changing. Over years, ATTR-CA had the most favourable outcome. NYHA functional class, syncope, BBs and ACE-i therapy were useful parameters for prognostic stratification. Further research is needed to investigate if they could be integrated in multiparametric scores for more accurate outcome prediction.

scholarly journals Concordance between quantitative and semi-quantitative analysis of Tc-99m-pyrophosphate scintigraphy for the diagnosis of transthyretin cardiac amyloidosis in patients with HFpEF

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Murat ◽  
H.E Yalvac ◽  
G.O Mert ◽  
I.A Sivrikoz ◽  
Y Cavusoglu
Keyword(s):  
Quantitative Analysis ◽  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Visual Assessment ◽  
Interatrial Septum ◽  
Left Ventricular ◽  
Myocardial Uptake ◽  
Cardiac Uptake ◽  
Funding Sources ◽  
American Society

Abstract Background Transthyretin (TTR) cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure with preserved ejection fraction (HFpEF). Cardiac scintigraphy with 99mTechnetium-pyrophosphate (99mTc-PYP) is referred as a simple, non-invasive and reliable method in the diagnosis of TTR-CA. American Society of Nuclear Cardiology Practice Points recommends two interpretative approaches: the quantitative heart-to-contralateral lung ratio (H/CL) at 1 hour or the semi-quantitative visual assessment at 3 hours after radiotracer injection. Purpose In this study, we evaluated the concordance between semi-quantitative and quantitative approaches in the diagnosis of TTR CA in patients with HFpEF. Methods This single-center, prospective study included 78 patients who had a diagnosis of HFpEF according to 2016 ESC HF guidelines. 99mTc-PYP cardiac scintigraphy was performed in 43 patients who have ≥2 red flags for TTR-CA including left ventricular hypertrophy (LVH) (wall thickness ≥12 mm), biventricular hypertrophy, sparkling pattern, reduction in longitudinal strain with apical sparing, thickening of the interatrial septum (>6mm), low-voltage, pseudo infarct pattern or atrioventricular block on ECG. In the absence of monoclonal protein in the serum and urine, Grade 2 to 3 myocardial uptake in semi-quantitative analysis at 3 hours or a H/CL ratio of ≥1.5 in quantitative analysis at 1 hour post injection of 99mTc-PYP is considered positive for TTR-CA. Grade 2–3 uptake with a H/CL ratio ≥1.5 or Grade 0–1 uptake with a H/CL ratio <1.5 were considered as concordant results. Grade 2–3 uptake with a H/CL ratio <1.5 or Grade 0–1 uptake with a H/CL ratio ≥1.5 were considered as discordant results. Results Mean age of study population was 68.26±9.97 years. 17 (39.5%) of 43 patients who underwent 99mTc-PYP cardiac scintigraphy showed a Grade 2 or 3 cardiac uptake and in these patients with Grade 2–3 uptake, 11 patients (65%) had a H/CL ratio ≥1.5 (concordant results) and 6 patients (35%) had a H/CL ratio <1.5 (discordant results). 26 (60.5%) of 43 patients showed Grade 0–1 cardiac uptake. All patients (100%) with Grade 0–1 uptake had a H/CL ratio <1.5 and therefore, showed concordant results. Overall, 37 (86%) patients had concordant and 6 (14%) patients had discordant results (Table 1). Conclusion The results of this study showed that although there was a high agreement between semi-quantitative and quantitative analysis of 99mTc-PYP cardiac scintigraphy, 14% of patients have discordant results and need further workup to confirm TTR-CA in patients with HFpEF. FUNDunding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Pfizer independent grant.

RESEARCH THE VALUE OF THE COMBINATION OF T WAVE ALTERNANS AND NT-PROBNT IN PREDICTING SUDDEN CARDIAC DEATH

2012 ◽  
pp. 74-83
Author(s):  
Anh Tien Hoang ◽  
Nhat Quang Nguyen
Keyword(s):  
Myocardial Infarction ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Healthy Person ◽  
Treadmill Test ◽  
T Wave ◽  
T Wave Alternans

Background: Decades of research now link TWA with inducible and spontaneous clinical ventricular arrhythmias. This bench-to-bedside foundation makes TWA, NT-ProBNP a very plausible index of susceptibility to ventricular arrythmia, and motivates the need to define optimal combination of TWA and NT-ProBNP in predicting ventricular arrythmia in myocardial infarction patients. We research this study with 2 targets: 1. To evaluate the role of TWA in predicting sudden cardiac death in myocardial infarction patients. 2. To evaluate the role of NT-ProBNP in predicting sudden cardiac death in myocardial infarction patients 3. Evaluate the role of the combined NT-ProBNP and TWA in predicting sudden cardiac death in myocardial infarction patients. Methods: Prospective study with follow up the mortality in 2 years: 71 chronic myocardial infarction patients admitted to hospital from 5/2009 to 5/20011 and 50 healthy person was done treadmill test to caculate TWA; ECG, echocardiography, NT-ProBNP. Results: Cut-off point of NT-ProBNP in predicting sudden cardiac death is 3168 pg/ml; AUC = 0,86 (95% CI: 0,72 - 0,91); Cut-off point of TWA in predicting sudden cardiac death is 107 µV; AUC = 0,81 (95% CI: 0,69 - 0,87); NT-ProBNP can predict sudden cardiac death with OR= 7,26 (p<0,01); TWA can predict sudden cardiac death with OR= 8,45 (p<0,01). The combined NT-ProBNP and TWA in predicting ventricular arrythmia in heart failure patients: OR= 17,91 (p<0,001). Conclusions: The combined NT-ProBNP and TWA have the best predict value of sudden cardiac death in myocardial infarction patients, compare to NT-ProBNP or TWA alone

scholarly journals P2.11-21 Usefulness of Diffusion-Weighted Whole-Body Imaging with Background Suppression in the Postoperative Follow-up Period

2019 ◽  
Vol 14 (10) ◽  
pp. S800
Author(s):  
K. Suemori ◽  
M. Kataoka ◽  
D. Okutani ◽  
T. Fujita ◽  
I. Togami ◽  
...  
Keyword(s):  
Whole Body ◽  
Background Suppression ◽  
Whole Body Imaging ◽  
Body Imaging ◽  
Diffusion Weighted

scholarly journals P014: Incidental findings in trauma whole-body CT scans: a systematic review

CJEM ◽  
2020 ◽  
Vol 22 (S1) ◽  
pp. S69-S69
Author(s):  
V. Tsang ◽  
K. Bao ◽  
J. Taylor
Keyword(s):  
Systematic Review ◽  
Incidental Findings ◽  
Traffic Accidents ◽  
Road Traffic ◽  
Subsequent Treatment ◽  
Whole Body ◽  
Trauma Patients ◽  
Patient Demographics ◽  
Study Results

Introduction: Whole-body computed tomography scans (WBCT) are a mainstay in the work-up of polytrauma or multiple trauma patients in the emergency department. While incredibly useful for identifying traumatic injuries, WBCTs also reveal incidental findings in patients, some of which require further diagnostic testing and subsequent treatment. Although the presence of incidental findings in WBCTs have been well documented, there has been no systematic review conducted to organize and interpret findings, determine IF prevalence, and document strategies for best management. Methods: A systematic review was conducted using MEDLINE, PUBMED, and EMBASE. Specific journals and reference lists were hand-mined, and Google Scholar was used to find any additional papers. Data synthesis was performed to gather information on patient demographics, prevalence and type of incidental findings (IFs), and follow-up management was collected. All documents were independently assessed by the two reviewers for inclusion and any disagreements were resolved by consensus. Results: 1231 study results were identified, 59 abstracts, and 12 included in final review. A mean of 53.9% of patients had at least one IF identified, 31.5% had major findings, and 68.5% had minor findings. A mean of 2.7 IFs per patient was reported for articles that included number of total IFs. The mean age of patients included in the studies were 44 years old with IFs more common in older patients and men with more IFs than women. IFs were most commonly found in the abdominal/pelvic region followed by kidneys. Frequency of follow-up documentation was poor. The most common reported mechanisms of injury for patients included in the study were MVA and road traffic accidents (60.0%) followed by falls from >3m (23.2%). Conclusion: Although there is good documentation on the mechanism of injury, patient demographics, and type of IF, follow-up for IFs following acute trauma admission lacks documentation and follow-up and is an identified issue in patient management. There is great need for systematic protocols to address management of IFs in polytrauma patients.

Regression of cardiac amyloid deposits with novel therapeutics: reaching new frontiers in cardiac ATTR amyloidosis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
L Chacko ◽  
A Martinez-Naharro ◽  
T Kotecha ◽  
R Martone ◽  
D Hutt ◽  
...  
Keyword(s):  
Treatment Group ◽  
Cardiac Amyloidosis ◽  
T1 Mapping ◽  
Group Versus ◽  
Control Group ◽  
Cardiac Amyloid ◽  
Attr Amyloidosis ◽  
Amyloid Load ◽  
The Impact

Abstract Background Cardiac involvement is the main driver of outcome in ATTR amyloidosis. Advances in therapeutics hold potential in transforming the course of the disease but the impact on cardiac amyloid load is unknown. The aim of this study was to evaluate the impact of patisiran, a new double stranded RNA based gene silencing therapy and a stabilizer, diflunisal, on cardiac amyloid load as measured by CMR and T1 mapping, in patients with ATTR amyloidosis. Methods and results Thirty-two patients with hereditary cardiac amyloidosis were studied. Sixteen patients received treatment with patisiran, and sixteen control subjects did not receive any disease modifying treatment. Patients were assessed with echocardiogram, CMR, NT-proBNP and six-minute walk time measurements at baseline and at 1 year (Mean interval 11.45±3.08 months in treatment group, mean interval 12.82±5.06 months in the control group). CMR analysis comprised LV volumes, T1 mapping to measure the extracellular volume (ECV) occupied by amyloid, T2 mapping and late gadolinium enhancement imaging. At 1-year follow-up, there was a substantial reduction in cardiac amyloid burden, in keeping with cardiac amyloid regression in 45% of patients on treatment. Overall the treatment group showed a reduction in ECV at 1 year follow up compared to an increase in ECV at 1 year in the control group (−1.37%, 95% CI: −3.43 to 0.68% versus 5.02%, 95% CI: 2.86% to 7.18% respectively, p&lt;0.001). The treatment group also showed an improvement in change in 6MWT at 1 year follow up compared to 6MWT at 1 year in the control group (−8.12 meters, 95% CI: −50.8 to 34.6 meters in the treatment group versus −132.27 meters, 95% CI: −216 to −48.6 meters in the control group, p=0.002). The treatment group showed a reduction in BNP at 1 year follow up compared to an increase in the control group (−567.87, 95% CI: −1288.90 to 153.15 in the treatment group versus 2004, 95% CI: 12.82 to 3995.45 in the control group, p&lt;0.001). There was no significant difference from baseline and 1-year data between the control and treatment groups for the difference in echocardiographic parameters, native T1, T2. There was a significant reduction in the percentage of injected dose by 99Tc-DPD scintigraphy in treated patients at 1 year compared to baseline. Conclusions These findings provide the first compelling evidence of substantial cardiac amyloid regression in ATTR amyloidosis, as well as the potential for CMR to be used to track response in treated patients with ATTR cardiac amyloidosis. Combination therapy with transthyretin knock down and stabilizing agents may well be synergistic given enhanced stoichiometry of stabilizers in the face of much reduced plasma transthyretin concentration. Funding Acknowledgement Type of funding source: None

scholarly journals Cardiac Amyloidosis with Discordant QRS Voltage between Frontal and Precordial Leads

Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Low Voltage ◽  
Disease Process ◽  
Left Ventricular ◽  
Clinical Suspicion ◽  
Immunoglobulin Light Chain ◽  
Different Types

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.

scholarly journals Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Henriette Schermacher Marstein ◽  
Kristin Godang ◽  
Berit Flatø ◽  
Ivar Sjaastad ◽  
Jens Bollerslev ◽  
...  
Keyword(s):  
Bone Turnover ◽  
Inflammatory Markers ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathy ◽  
Disease Onset ◽  
Whole Body ◽  
Mineral Density ◽  
Z Scores

Abstract Background Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children and adolescents. Both the disease and its treatment with glucocorticoids may negatively impact bone formation. In this study we compare BMD in patients (children/adolescence and adults) with long-standing JDM with matched controls; and in patients, explore how general/disease characteristics and bone turnover markers are associated with BMD. Methods JDM patients (n = 59) were examined median 16.8y (range 6.6–27.0y) after disease onset and compared with 59 age/sex-matched controls. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD of the whole body and lumbar spine (spine) in all participants, and of ultra-distal radius, forearm and total hip in participants ≥20y only. Markers of bone turnover were analysed, and associations with outcomes explored. Results Reduced BMD Z-scores (<−1SD) were found in 19 and 29% of patients and 7 and 9% of controls in whole body and spine, respectively (p-values < 0.05). BMD and BMD Z-scores for whole body and spine were lower in all patients and for < 20y compared with their respective controls. In participants ≥20y, only BMD and BMD Z-score of forearm were lower in the patients versus controls. In patients, BMD Z-scores for whole body and/or spine were found to correlate negatively with prednisolone use at follow-up (yes/no) (age < 20y), inflammatory markers (age ≥ 20y) and levels of interferon gamma-induced protein 10 (IP-10) (both age groups). In all patients, prednisolone use at follow-up (yes/no) and age ≥ 20y were independent correlates of lower BMD Z-scores for whole body and spine, respectively. Conclusion In long-term JDM, children have more impairment of BMD than adults in spine and whole-body. Associations with BMD were found for both prednisolone and inflammatory markers, and a novel association was discovered with the biomarker of JDM activity, IP-10.

Regional cardiac uptake of 99-Tc-DPD is a novel powerful and independent prognostic marker in cardiac ATTR wild type amyloidosis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Milani ◽  
G Cavenaghi ◽  
L Obici ◽  
R Mussinelli ◽  
C Klersy ◽  
...  
Keyword(s):  
Prognostic Value ◽  
Cox Regression ◽  
Expectation Maximization Algorithm ◽  
Linear Increase ◽  
Whole Body ◽  
Wall Region ◽  
Cardiac Spect ◽  
Risk Of Death ◽  
Cardiac Wall

Abstract Background Skeletal scintigraphy with bone tracers is a key tool for cardiac ATTR diagnosis. However its prognostic value has not been systematically assessed. Purpose We evaluated the prognostic relevance of a quantitative method to assess regional 99mTc-DPD uptake by SPECT in the heart of ATTRwt patients. Methods All ATTRwt patients (n=229) undergoing clinical assessment and bone scintigraphy at our center (from 2012 to 2019) were enrolled. Theyreceived approximately 700 MBq of 99mTc-DPD. Planar whole body acquisition 10' after the injection followed by cardiac SPECT after 3 hours were performed. SPECT data were reconstructed into 64x64 matrices with an ordered-subset expectation maximization algorithm. For each wall region and for the apex, a circular region of interest (ROI, 20 pixels) was manually drawn and a value equating to the number of counts contained in the ROI was obtained. Partial correlation of ln-transformed ROI and biomarkers was retrieved from a multivariable regression model, while controlling for each cardiac wall region. Multivariable Cox regression was used to assess the prognostic role of lnROI while adjusting for wall region, NT-proBNP, cTnI and eGFR. Hazard ratios and 95% confidence intervals (HR, 95% CI) were computed. The Harrell's c statistic was reported for model discrimination. The interaction of biomarker and regional wall on survival was assessed; also, to account for intra-subject correlation of measures, within subject robust standard errors were computed. Results Median follow-up was 21 months (IQR 11, 40) and 39 (17%) patients died. Median age was 76 years (IQR, 72–80), NT-proBNP 2944 ng/L (IQR, 1815–5319), cTnI 0.095 ng/L (IQR, 0.062–0.144) and eGFR 62 mL/min (IQR, 51–77). ROI did not correlate with any of NT-proBNP, eGFR, age, cTnI or mLVWT (R&lt;1% in all cases). All analyses were adjusted for cardiac wall. At the multivariable Cox regression (Harrell's c=0.75), there was a linear increase in the risk of death associated with lnROI (HR 2.14, P=0.014), which was independent of cardiac wall region, NTproBNP, cTnI and eGFR. Only cTnI maintained a significant prognostic value. The association of lnROI and mortality was not modified by the site of measurement test for interaction with cardiac wall p=0.818). At the predefined subgroup analysis, the risk of death was similar for all walls; we computed the optimal cut-off for 12 months survival at the apex (a region usually lately involved) to 4193 (AUC: 0.68, sensitivity 80%, specificity 68%). At the multivariable Cox regression (Harrell's c 0.76), apex ROI&gt;4193 was an independent predictor of death (HR 3.60, 95% CI 1.45–8.93, p=0.006) and outperformed all the biomarkers tested. Conclusions Quantitative assessment of ROI uptake at cardiac SPECT is a powerful predictor of survival in ATTRwt patients, independent of and outperforming the other known prognostic factors. This observation warrants validation with prolonged follow-up and in independent patient series. Funding Acknowledgement Type of funding source: None

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