(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses.
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TLDR
In this paper, the discriminatory ability of 99mTc-pyrophosphate scintigraphy in immunoglobulin light chain (AL) versus transthyretin-related cardiac amyloidoses (ATTR) was validated.Abstract:
Background— Differentiating immunoglobulin light-chain (AL) from transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, therapy, and genetic counseling. We validated the discriminatory ability of 99mTc-pyrophosphate (99mTc-PYP) scintigraphy in AL versus ATTR.
Methods and Results— Forty-five subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) underwent 99mTc-PYP planar and single-photon positive emission computed tomography cardiac imaging. Scans were performed by experienced nuclear cardiologists blinded to the subjects’ cohort assignment. Cardiac retention was assessed with both a semiquantitative visual score (range, 0; no uptake to 3, diffuse uptake) and by quantitative analysis by drawing a region of interest over the heart corrected for contralateral counts and calculating a heart-to-contralateral ratio. Subjects with ATTR cardiac amyloid had a significantly higher semiquantitative cardiac visual score than the AL cohort (2.9±0.06 versus 0.8±0.27; P 1.5 consistent with intensely diffuse myocardial tracer retention had a 97% sensitivity and 100% specificity with area under the curve 0.992, P <0.0001 for identifying ATTR cardiac amyloidosis.
Conclusions— 99mTc-PYP cardiac imaging distinguishes AL from ATTR cardiac amyloidosis and may be a simple, widely available method for identifying subjects with ATTR cardiac amyloidosis, which should be studied in a larger prospective manner.read more
Citations
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Journal ArticleDOI
Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis.
Julian D. Gillmore,Mathew S. Maurer,Rodney H. Falk,Giampaolo Merlini,Thibaud Damy,Angela Dispenzieri,Ashutosh D. Wechalekar,John L. Berk,John L. Berk,Candida Cristina Quarta,Martha Grogan,Helen J. Lachmann,Sabahat Bokhari,Adam Castano,Sharmila Dorbala,Geoff B. Johnson,Andor W. J. M. Glaudemans,Tamer Rezk,Marianna Fontana,Giovanni Palladini,Paolo Milani,P.L Guidalotti,Katarina Flatman,Thirusha Lane,Frederick W. Vonberg,Carol J. Whelan,James C. Moon,Frederick L. Ruberg,Frederick L. Ruberg,Edward J. Miller,David F. Hutt,Bouke P. C. Hazenberg,Claudio Rapezzi,Philip N. Hawkins +33 more
TL;DR: Bone scintigraphy enables the diagnosis of cardiac ATTR amyloidosis to be made reliably without the need for histology in patients who do not have a monoclonal gammopathy, and proposes noninvasive diagnostic criteria that are applicable to the majority of patients with this disease.
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Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction
Esther Gonzalez-Lopez,María Gallego-Delgado,Gonzalo Guzzo-Merello,F. Javier de Haro-del Moral,Marta Cobo-Marcos,Carolina Robles,Belén Bornstein,Clara Salas,Enrique Lara-Pezzi,Luis Alonso-Pulpón,Pablo García-Pavía +10 more
TL;DR: ATTRwt is an underdiagnosed disease that accounts for a significant number (13%) of HFpEF cases and the effect of emerging TTR-modifying drugs should be evaluated in these patients.
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2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society
Fred M. Kusumoto,Mark H. Schoenfeld,Coletta Barrett,James R. Edgerton,Kenneth A. Ellenbogen,Michael R. Gold,Nora Goldschlager,Robert M. Hamilton,Jose A. Joglar,Robert Kim,Richard T. Lee,Joseph E. Marine,Christopher J. McLeod,Keith R. Oken,Kristen K. Patton,Cara N. Pellegrini,Kimberly A. Selzman,Annemarie Thompson,Paul D. Varosy +18 more
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Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review
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Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement.
Adam Castano,David L. Narotsky,Nadira Hamid,Omar K. Khalique,Rachelle Morgenstern,Albert DeLuca,Jonah Rubin,Codruta Chiuzan,Tamim Nazif,Torsten Vahl,Isaac George,Susheel Kodali,Martin B. Leon,Rebecca T. Hahn,Sabahat Bokhari,Mathew S. Maurer +15 more
TL;DR: Transthyretin cardiac amyloidosis is prevalent in 16% of patients with severe calcific AS undergoing TAVR and is associated with a severe AS phenotype of low-flow low-gradient with mildly reduced ejection fraction.
References
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Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens.
Julie A. Vrana,Jeffrey D. Gamez,Benjamin J. Madden,Jason D. Theis,H. Robert Bergen,Ahmet Dogan +5 more
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Noninvasive Etiologic Diagnosis of Cardiac Amyloidosis Using 99mTc-3,3-Diphosphono-1,2-Propanodicarboxylic Acid Scintigraphy
Enrica Perugini,Pier Luigi Guidalotti,Fabrizio Salvi,Robin M. T. Cooke,Cinzia Pettinato,Letizia Riva,Ornella Leone,Mohsen Farsad,Paolo Ciliberti,Letizia Bacchi-Reggiani,Francesco Fallani,Angelo Branzi,Claudio Rapezzi +12 more
TL;DR: It is concluded that 99mTc-DPD scintigraphy is a useful step in the workup of the differential diagnosis of TTR versus AL etiology in patients with documented cardiac amyloidosis.
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Systemic Cardiac Amyloidoses Disease Profiles and Clinical Courses of the 3 Main Types
Claudio Rapezzi,Giampaolo Merlini,Candida Cristina Quarta,Letizia Riva,Simone Longhi,Ornella Leone,Fabrizio Salvi,Paolo Ciliberti,Francesca Pastorelli,Elena Biagini,Fabio Coccolo,Robin M. T. Cooke,Letizia Bacchi-Reggiani,Diego Sangiorgi,Alessandra Ferlini,Michele Cavo,Elena Zamagni,Maria Luisa Fonte,Giovanni Palladini,Francesco Salinaro,Francesco Musca,Laura Obici,Angelo Branzi,Stefano Perlini +23 more
TL;DR: AL, ATTRm, and ATTRwt should be considered 3 different cardiac diseases, probably characterized by different pathophysiological substrates and courses.
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Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis.
Helen J. Lachmann,David R. Booth,SE Booth,A Bybee,Janet A. Gilbertson,Julian D. Gillmore,Mark B. Pepys,Philip N. Hawkins +7 more
TL;DR: A genetic cause should be sought in all patients with amyloidsosis that is not the reactive systemic amyloid A type and in whom confirmation of the light-chain (AL) type cannot be obtained.
Journal ArticleDOI
Variant-sequence transthyretin (isoleucine 122) in late-onset cardiac amyloidosis in black Americans
Daniel R. Jacobson,Raymond D. Pastore,Robert Yaghoubian,Immaculata Kane,Gloria Gallo,Francis S. Buck,Joel N. Buxbaum +6 more
TL;DR: The assessment of elderly black patients with unexplained heart disease should include a consideration of transthyretin amyloidosis, particularly that related to the Ile 122 allele.
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