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Utility of ^{99 m}Tc-Pyrophosphate Scintigraphy in Diagnosing Transthyretin Cardiac Amyloidosis in Real-World Practice.

ABSTRACT: Background: Amyloid transthyretin (ATTR) cardiac amyloidosis has now been recognized as one of the major causes of heart failure, especially in elderly patients. The purpose of the present study was to validate the usefulness of technetium-99 m (^{99 m}Tc)-pyrophosphate (^{99 m}Tc-PYP) scintigraphy in the screening diagnosis for ATTR amyloidosis in daily clinical practice. Methods?and?Results: Ninety-eight patients underwent ^{99 m}Tc-PYP scintigraphy in the previous 3 years (PYP positive/negative, 18/80), of whom 29 underwent concomitant endomyocardial biopsy (ATTR positive/negative, 9/20). The sensitivity and specificity of ^{99 m}Tc-PYP scintigraphy for the diagnosis of biopsy-proven ATTR amyloidosis were 0.889 and 0.950, respectively. Age, gender, N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level, or electrocardiogram findings did not differ significantly between PYP-positive and PYP-negative patients. Left ventricular (LV) wall thickness was significantly greater in PYP-positive than in PYP-negative patients, but LV ejection fraction or prevalence of atrial fibrillation was similar between groups. In the PYP-positive patients, higher uptake of PYP correlated with younger age and lower NT-proBNP. Conclusions: ^{99 m}Tc-PYP scintigraphy was useful, with high sensitivity and specificity in the screening diagnosis for ATTR cardiac amyloidosis, which is difficult to diagnose on clinical characteristics alone. ^{99 m}Tc-PYP scintigraphy should be considered to elucidate the underlying causes of heart failure, especially in elderly patients based on the higher prevalence of ATTR cardiac amyloidosis in this population.

SUBMITTER: Tsutsui Y

PROVIDER: S-EPMC7892493 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Utility of 99 mTc-Pyrophosphate Scintigraphy in Diagnosing Transthyretin Cardiac Amyloidosis in Real-World Practice.

Tsutsui Yoshitomo Y Kubota Toru T Kato Seiya S Nozoe Masatsugu M Suematsu Nobuhiro N Okabe Masanori M Yamamoto Yusuke Y Tsutsui Hiroyuki H

Circulation reports 20190614 7

Background: Amyloid transthyretin (ATTR) cardiac amyloidosis has now been recognized as one of the major causes of heart failure, especially in elderly patients. The purpose of the present study was to validate the usefulness of technetium-99 m (99 mTc)-pyrophosphate (99 mTc-PYP) scintigraphy in the screening diagnosis for ATTR amyloidosis in daily clinical practice. Methods and Results: Ninety-eight patients underwent 99 mTc-PYP scint ...[more]

PMID: 33693151

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Project description:ObjectivesThe purpose of this study was to examine the diagnostic value of planar 99mTc-pyrophosphate (PYP) imaging at 1 and 3 h after tracer administration in patients with suspected transthyretin cardiac amyloidosis (ATTR-CA) using SPECT as a reference standard. We also tested whether blood pool activity of PYP is associated with renal dysfunction.MethodsPYP images of 109 consecutive patients with suspected ATTR-CA were retrospectively reviewed. The myocardial PYP uptake was visually graded on a scale of 0 to 3 and quantified with the heart-to-contralateral (H/CL) ratio in accordance with the current expert consensus recommendations. The diagnostic value of planar images for identifying positive PYP SPECT was assessed by a receiver-operating characteristic curve analysis with the area under the curve (AUC). The uptake ratios of the ascending and descending aorta, left atrium, and trapezius muscle divided by the liver uptake were measured on SPECT images and compared to the renal function.ResultsA total of 41 patients (38%) had myocardial PYP uptake on SPECT images. In comparison with the visual scores on 1-h anterior planar images, those on 3-h anterior planar images had lower sensitivity (80.5% vs. 97.6%) and higher specificity (86.8% vs. 55.9%) for identifying positive PYP SPECT. The ROC analysis showed that the combination of visual scores on both 1-h and 3-h anterior planar images had significantly higher AUC values in comparison with 1-h anterior planar images alone (0.90 [95% CI 0.83-0.94] vs. 0.83 [95% CI 0.75-0.88]; P < 0.001), which was comparable to the AUC values on 3-h anterior planar images alone (0.88 [95% CI 0.80-0.92]; P = 0.071). In comparison with visual scores on 1-h or 3-h anterior planar images alone, the combination of visual scores and H/CL ratio did not significantly improve the diagnostic value for identifying positive PYP SPECT (P = 0.73 and P = 0.50, respectively). The uptake ratios of ascending aorta/liver, descending aorta/liver, left atrium/liver, and trapezius muscle/liver were not significantly associated with the serum creatinine level or estimated glomerular filtration rate (P > 0.05 for all).ConclusionsIn the assessment of ATTR-CA using PYP imaging, visual scores on 3-h anterior planar images for identifying positive PYP SPECT had lower sensitivity and higher specificity in comparison with those on 1-h anterior planar images. The diagnostic value of the visual scores on 1-h and 3-h anterior planar images was not improved by adding the H/CL ratio. Blood pool activity of PYP was not significantly associated with renal dysfunction.

Project description:PurposeAs an autoimmune inflammatory disorder, active thyroid-associated ophthalmopathy (TAO) is managed optimally by immunosuppression. In this study, we aimed to evaluate octreotide scintigraphy and the level of serum extraocular muscle antibodies in TAO activity.Patients and methodsThis prospective study comprised 304 patients with active TAO (the clinical activity score; CAS?3), 73 with inactive TAO (CAS<3), 128 with Graves' disease (GD) without ophthalmopathy, and 100 healthy subjects. Moderate-to-severe active TAO patients (CAS?5) received intravenous injection of methylprednisolone; mild active patients (3?CAS?4) received periocular injection of triamcinolone acetonide. 99Tcm-octreotide scintigraphy and serum levels of calsequestrin, uveal auto-antigen with coiled-coil domains and ankyrin repeats (UACA) and G2s antibodies were detected before and after treatment.Results99Tcm-octreotide scintigraphy was positive in active TAO patients (97%) with elevated uptake ratio (UR) (P<0.05), and showed a significant correlation with CAS (r=0.816, P<0.01). After treatment both UR and CAS decreased significantly (P<0.05). The receiving operator characteristic curve (ROC) showed that the best UR threshold for discriminating active and inactive TAO was 1.34 (sensitivity, 100%; specificity, 89.4%). The level of serum calsequestrin antibody was higher in active TAO (P<0.05), showed a significant correlation with CAS (r=0.738, P<0.05), and also decreased after treatment (P<0.05). The best serum calsequestrin antibody threshold of the ROC curve was 138?ng/l (sensitivity, 88.4%; specificity, 89.2%). The UACA antibody was elevated in both TAO and GD patients (P<0.05), with no significant difference (P>0.05). As to G2s, no significant difference was found between all groups (P>0.05). Moreover, six GD patients (4.69%) with elevated calsequestrin developed active TAO 12 weeks later.Conclusion99Tcm-octreotide scintigraphy played a critical role in the evaluation of the clinical activity and therapeutic efficacy of TAO. Autoimmunity against calsequestrin in the pathogenesis of the eye muscle components may provide further objective evidence of myopathy in active TAO. Furthermore, calsequestrin antibody may predict myopathy in active TAO.

Project description:BackgroundEchocardiographic deformation-based ratios and novel multi-parametric scores have been suggested to discriminate transthyretin cardiac amyloidosis (ATTR-CM) from other causes of increased left ventricular wall thickness among patients referred for ATTR-CM evaluation. Their relative predictive accuracy has not been well studied. We sought to (1) identify echocardiographic parameters predictive of ATTR-CM and (2) compare the diagnostic accuracy of these parameters in patients with suspected ATTR-CM referred for technetium-99m-pyrophosphate scintigraphy.MethodsEchocardiograms from 598 patients referred to 3 major amyloidosis centers for technetium-99m-pyrophosphate to detect ATTR-CM were analyzed, including longitudinal strain (LS) analysis. Deformation ratios (septal apex to base ratio, relative apical sparing, ejection fraction to global LS), a multi-center European increased wall thickness score, and Mayo Clinic derived ATTR score (transthyretin cardiac amyloidosis score) were calculated. A logistic regression model was used to identify the parameters that best associated with a diagnosis of ATTR-CM. Comparison of the diagnostic capacity of the parameters was performed by receiver operating characteristic curves and the area under the curve (AUC).ResultsOver half of the subjects (54.2%) were diagnosed with ATTR-CM (78% were men, median age of 76 years). Age, inferolateral wall thickness, and basal LS were the strongest predictors of ATTR-CM, AUC of 0.87 (95% CI: 0.83, 0.90), superior to the increased wall thickness score AUC of 0.78 (95% CI: 0.73, 0.83; P=0.004). An inferolateral wall thickness of ≥14 mm (AUC: 0.73) was as accurate as the published cut-offs for transthyretin cardiac amyloidosis score and septal apex to base (AUC: 0.72 and 0.69, P=0.8 and P=0.1, respectively), and was superior to ejection fraction to global LS and relative apical sparing (AUC: 0.64 and 0.53, P<0.001, respectively). A cut-off of ≥-8% for average basal LS (AUC: 0.76, CI: 0.72-0.79) had a similar area under the curve to transthyretin cardiac amyloidosis score (TCAS) (P=0.2); outperforming the other indices (P<0.01).ConclusionInferolateral wall thickness and average basal LS performed as well as or better than more complex echo ratios and multiparametric scores to predict ATTR-CM.

Dataset Information

Utility of ^{99 m}Tc-Pyrophosphate Scintigraphy in Diagnosing Transthyretin Cardiac Amyloidosis in Real-World Practice.

Publications

Utility of <sup>99 m</sup>Tc-Pyrophosphate Scintigraphy in Diagnosing Transthyretin Cardiac Amyloidosis in Real-World Practice.

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