National patterns in imaging utilization for diagnosis of cardiac amyloidosis: A focus on Tc99m-pyrophosphate scintigraphy

Harb, Serge C.; Haq, Muhammad; Flood, Kathleen; Guerrieri, A. M.; Passerell, Wendy; Jaber, Wael A.; Miller, Edward J.

doi:10.1007/s12350-016-0478-3

Cited by 33 publications

(23 citation statements)

References 7 publications

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“…Late gadolinium enhancement imaging is highly sensitive and specific with images virtually diagnostic for amyloidosis 53 . The use of radionuclide imaging of the heart with Tc PYP or DPD has been found useful in distinguishing AL cardiac amyloidosis (no uptake) from TTR cardiac amyloidosis (2+ or greater uptake) 54 …”

Section: Prognosismentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Gertz

2020

American J Hematol

114

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Disease Overview Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical smoldering multiple myeloma or monoclonal gammopathy undetermined significance (MGUS).” Diagnosis Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy. Prognosis N‐terminal pro‐brain natriuretic peptide (NT‐proBNP), serum troponin T, and difference between involved and uninvolved immunoglobulin free light chain (FLC) values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months. Therapy All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include systolic blood pressure >90 mmHg, troponin T < 0.06 ng/mL and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered cyclophosphamide‐bortezomib‐dexamethasone or daratumumab‐containing regimens as it appears to be highly active in AL amyloidosis. Future Challenges Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end‐stage organ failure.

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Section: Prognosismentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Gertz

2020

American J Hematol

114

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“…Late gadolinium enhancement imaging is highly sensitive and specific with images virtually diagnostic for amyloidosis . The use of radionuclide imaging of the heart with Tc PYP or DPD has been found useful in distinguishing AL cardiac amyloidosis (no uptake) from TTR cardiac amyloidosis (2+ or greater uptake) .…”

Section: Prognosismentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2016 update on diagnosis, prognosis, and treatment

Gertz

2016

American J Hematol

107

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All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure of involved sites. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include systolic blood pressure >90 mmHg, troponin T <0.06 ng mL21, age <70 years, and serum creatinine 1.7 mg dL21. Nontransplant candidates can be offered melphalan-dexamethasone or cyclophosphamide-bortezomib-dexamethasone. Other combinations of chemotherapy with agents such as cyclophosphamide-thalidomide (or lenalidomide)-dexamethasone, bortezomib-dexamethasone, and melphalan-prednisone-lenalidomide have documented activity. Antibodies designed to dissolve existing amyloid deposits are under study for previously treated and untreated patients. Late diagnosis remains a major obstacle to initiating effective therapy. Am. J. Hematol., 2016. © 2016 Wiley Periodicals, Inc. Am. J. Hematol. 91:948-956, 2016. © 2016 Wiley Periodicals, Inc.

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“…9,10 On the other hand, in the United States, following the work by Bokhary et al, the most commonly used method is the heart-to-contralateral lung ratio (H/ CL), obtained by drawing a region of interest (ROI) over the heart corrected for a contralateral ROI counts. 6,13 To the best of our knowledge, no comprehensive clinical comparison of different semi-quantitative indices in amyloid identification has been till now performed.…”

Section: H/wbrmentioning

confidence: 99%

Semi-quantitative indices of cardiac uptake in patients with suspected cardiac amyloidosis undergoing 99mTc-HMDP scintigraphy

Gallini

Tutino

Martone

et al. 2021

Journal of Nuclear Cardiology

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Background. 99mTc-HMDP scintigraphy has proved its efficacy in non-invasive diagnosis of cardiac amyloidosis (CA) and is currently interpreted according to the Perugini qualitative assessment. Several semi-quantitative indices have been proposed to overcome inherent possible limitations of visual grading. Our aim was to comparatively evaluate six different indices and their diagnostic performance. Methods. We retrospectively reviewed scintigraphy of 76 patients (53 ATTR, 12 AL, 11 LVH) who underwent diagnostic evaluation at our centre. ROC-curve analysis was performed to identify optimal cutoff and relative diagnostic accuracy of six different indices (of which one was proposed for the first time), both in identifying CA patients and in discriminating patients according to their Perugini score. Results. Heart/Whole-body ratios proved to be the most accurate (100%) in identifying CA patients. Heart/Pelvis ratio (with soft tissue background correction) offered acceptable accuracy (98%), with the largest area under the curve (AUC) (0.98) in discriminating patients with Perugini ‡ 2. Heart/Contralateral Lung ratio confirmed to be exposed to confounding background noise in case of simultaneous lung uptake. Heart/Skull ratio had the worst performance, with six false-negative patients in ATTR identification. Conclusion. Heart/Whole-body ratios may be robust and effective semi-quantitative indices for the evaluation of CA by means of scintigraphy (J Nucl Cardiol 2019

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National patterns in imaging utilization for diagnosis of cardiac amyloidosis: A focus on Tc99m-pyrophosphate scintigraphy

Abstract: This national survey shows relatively low penetrance and high variability in Tc99m-PYP scintigraphy for CA diagnosis highlighting the need for standardization.

Cited by 33 publications

References 7 publications

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment

Immunoglobulin light chain amyloidosis: 2016 update on diagnosis, prognosis, and treatment

Semi-quantitative indices of cardiac uptake in patients with suspected cardiac amyloidosis undergoing 99mTc-HMDP scintigraphy

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