Dataset Information

Technetium-99m pyrophosphate cardiac SPECT in endomyocardial biopsy negative cardiac amyloidosis.

ABSTRACT: Cardiac amyloidosis is an under-appreciated cause of heart failure. Establishing a diagnosis is important because traditional heart failure treatment regimens can worsen left ventricular failure in this disease. Endomyocardial biopsy is the gold standard for diagnosis; however, scintigraphy with radiolabeled phosphate derivatives and cardiac magnetic resonance imaging have been shown to have high sensitivity and specificity in diagnosing cardiac amyloidosis. Furthermore, cardiac scintigraphy can reliably differentiate amyloid subtypes. We present a case of transthyretin-related cardiac amyloidosis with a negative endomyocardial biopsy but positive 99m-technetium pyrophosphate single photon emission computed tomography scan and cardiac magnetic resonance imaging. We discuss the utility of 99m-technetium pyrophosphate imaging in cardiac amyloidosis and the role of single photon emission computed tomography. Finally, we review the several forms of cardiac amyloidosis and how they pertain to cardiac scintigraphy.

SUBMITTER: Krupa M

PROVIDER: S-EPMC6073076 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature

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Technetium-99m pyrophosphate cardiac SPECT in endomyocardial biopsy negative cardiac amyloidosis.

Krupa Martin M Nguyen Rina R Revels Jonathan J Johnson Lester S LS

Radiology case reports 20180719 5

Cardiac amyloidosis is an under-appreciated cause of heart failure. Establishing a diagnosis is important because traditional heart failure treatment regimens can worsen left ventricular failure in this disease. Endomyocardial biopsy is the gold standard for diagnosis; however, scintigraphy with radiolabeled phosphate derivatives and cardiac magnetic resonance imaging have been shown to have high sensitivity and specificity in diagnosing cardiac amyloidosis. Furthermore, cardiac scintigraphy can ...[more]

PMID: 30093927

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Project description:ObjectivesThis study prospectively evaluated endomyocardial biopsies in patients with heart failure with preserved ejection fraction (HFpEF) to identify histopathologic phenotypes and their association with clinical characteristics.BackgroundMyocardial tissue analysis from a prospectively defined HFpEF cohort reflecting contemporary comorbidities is lacking.MethodsPatients with HFpEF (EF ≥50%) referred to the Johns Hopkins HFpEF Clinic between August 2014 and September 2018 were enrolled for right heart catheterization and endomyocardial biopsy. Clinical features, echocardiography, hemodynamics, and tissue histology were determined and compared with controls (unused donor hearts) and HF with reduced EF (HFrEF).ResultsOf the 108 patients enrolled, median age was 66 years (25th to 75th percentile: 57 to 74 years), 61% were women, 57% were African American, 62% had a previous HF hospitalization, median systolic blood pressure was 141 mm Hg (25th to 75th percentile: 125 to 162 mm Hg), body mass index (BMI) was 37 kg/m2 (25th to 75th percentile: 32 to 45 kg/m2), and 97% were on a loop diuretic. Myocardial fibrosis and myocyte hypertrophy were often present (93% and 88%, respectively); however, mild in 71% with fibrosis and in 52% with hypertrophy. Monocyte infiltration (CD68+ cells/mm2) was greater in patients with HFpEF versus controls (60.4 cells/mm2 [25th to 75th percentile: 36.8 to 97.8] vs. 32.1 cells/mm2 [25th to 75th percentile: 22.3 to 59.2]; p = 0.02) and correlated with age and renal disease. Cardiac amyloidosis (CA) was diagnosed in 15 (14%) patients (HFpEF-CA: 7 patients with wild-type transthyretin amyloidosis [ATTR], 4 patients with hereditary ATTR, 3 patients with light-chain amyloidosis, and 1 patient with AA (secondary) amyloidosis), of which 7 cases were unsuspected. Patients with HFpEF-CA were older, with lower BMI, higher left ventricular mass index, and higher N-terminal pro-B-type natriuretic peptide and troponin I levels.ConclusionsIn this large, prospective myocardial tissue analysis of HFpEF, myocardial fibrosis and hypertrophy were common, CD68+ inflammation was increased, and CA prevalence was 14%. Tissue analysis in HFpEF might improve precision therapies by identifying relevant myocardial mechanisms.

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.

Dataset Information

Technetium-99m pyrophosphate cardiac SPECT in endomyocardial biopsy negative cardiac amyloidosis.

Publications

Technetium-99m pyrophosphate cardiac SPECT in endomyocardial biopsy negative cardiac amyloidosis.

Similar Datasets

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