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Article ; Online: Comparison of

Somers, Henry / Tanzim, Ubaid / Porcari, Aldostefano / Razvi, Yousuf / Rezk, Tamer / Patel, Rishi / Chacko, Liza / Rowczenio, Dorota / Gilbertson, Janet A / Hutt, David F / Hawkins, Philip N / Fontana, Marianna / Gillmore, Julian D

JACC. Cardiovascular imaging

2022 Volume 15, Issue 7, Page(s) 1353–1355

MeSH term(s)	Amyloid Neuropathies, Familial/diagnostic imaging ; Amyloid Neuropathies, Familial/genetics ; Cardiomyopathies/etiology ; Cardiomyopathies/genetics ; Echocardiography ; Humans ; Prealbumin/genetics ; Predictive Value of Tests ; Radionuclide Imaging ; Radiopharmaceuticals
Chemical Substances	Prealbumin ; Radiopharmaceuticals
Language	English
Publishing date	2022-05-11
Publishing country	United States
Document type	Letter
ZDB-ID	2491503-8
ISSN	1876-7591 ; 1936-878X
ISSN (online)	1876-7591
ISSN	1936-878X
DOI	10.1016/j.jcmg.2022.02.016
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Article ; Online: Unusual imaging findings in a patient with AL amyloidosis.

Hutt, David F / Quigley, Anne-Marie / Wechalekar, Ashutosh D

British journal of haematology

2015 Volume 170, Issue 3, Page(s) 289

MeSH term(s)	Aged ; Amyloidosis/diagnostic imaging ; Female ; Humans ; Radiography ; Radionuclide Imaging
Language	English
Publishing date	2015-08
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/bjh.13491
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Article ; Online: (99m)Tc-DPD scintigraphy as a novel imaging modality for identification of skeletal muscle amyloid deposition in light-chain amyloidosis.

Hutt, David F / Gilbertson, Janet / Quigley, Anne-Marie / Wechalekar, Ashutosh D

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

2016 Volume 23, Issue 2, Page(s) 134–135

MeSH term(s)	Aged ; Amyloidosis/diagnostic imaging ; Amyloidosis/metabolism ; Amyloidosis/mortality ; Amyloidosis/pathology ; Cardiomyopathies/diagnostic imaging ; Cardiomyopathies/metabolism ; Cardiomyopathies/mortality ; Cardiomyopathies/pathology ; Creatine Kinase/blood ; Diphosphonates ; Humans ; Male ; Middle Aged ; Muscle, Skeletal/metabolism ; Muscle, Skeletal/pathology ; Natriuretic Peptide, Brain/blood ; Organotechnetium Compounds ; Peptide Fragments/blood ; Radionuclide Imaging/methods ; Serum Amyloid P-Component/metabolism ; Survival Analysis ; Troponin/blood
Chemical Substances	Diphosphonates ; Organotechnetium Compounds ; Peptide Fragments ; Serum Amyloid P-Component ; Troponin ; pro-brain natriuretic peptide (1-76) ; technetium Tc 99m 1,1-diphosphonopropane-2,3-dicarboxylic acid ; Natriuretic Peptide, Brain (114471-18-0) ; Creatine Kinase (EC 2.7.3.2)
Language	English
Publishing date	2016-06
Publishing country	England
Document type	Journal Article
ZDB-ID	1205246-2
ISSN	1744-2818 ; 1350-6129
ISSN (online)	1744-2818
ISSN	1350-6129
DOI	10.3109/13506129.2016.1158160
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Zs.A 4114: Show issues

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Article ; Online: Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping.

JACC. Cardiovascular imaging

2023 Volume 16, Issue 8, Page(s) 1038–1052

Abstract: Background: Systemic light chain amyloidosis is a multisystem disorder that commonly involves the heart, liver, and spleen. Cardiac magnetic resonance with extracellular volume (ECV) mapping provides a surrogate measure of the myocardial, liver, and ... ...

Abstract	Background: Systemic light chain amyloidosis is a multisystem disorder that commonly involves the heart, liver, and spleen. Cardiac magnetic resonance with extracellular volume (ECV) mapping provides a surrogate measure of the myocardial, liver, and spleen amyloid burden. Objectives: The purpose of this study was to assess multiorgan response to treatment using ECV mapping, and assess the association between multiorgan treatment response and prognosis. Methods: The authors identified 351 patients who underwent baseline serum amyloid-P-component (SAP) scintigraphy and cardiac magnetic resonance at diagnosis, of which 171 had follow-up imaging. Results: At diagnosis, ECV mapping demonstrated that 304 (87%) had cardiac involvement, 114 (33%) significant hepatic involvement, and 147 (42%) significant splenic involvement. Baseline myocardial and liver ECV independently predict mortality (myocardial HR: 1.03 [95% CI: 1.01-1.06]; P = 0.009; liver HR: 1.03; [95% CI: 1.01-1.05]; P = 0.001). Liver and spleen ECV correlated with amyloid load assessed by SAP scintigraphy (R = 0.751; P < 0.001; R = 0.765; P < 0.001, respectively). Serial measurements demonstrated ECV correctly identified changes in liver and spleen amyloid load derived from SAP scintigraphy in 85% and 82% of cases, respectively. At 6 months, more patients with a good hematologic response had liver (30%) and spleen (36%) ECV regression than myocardial regression (5%). By 12 months, more patients with a good response demonstrated myocardial regression (heart 32%, liver 30%, spleen 36%). Myocardial regression was associated with reduced median N-terminal pro-brain natriuretic peptide (P < 0.001), and liver regression with reduced median alkaline phosphatase (P = 0.001). Changes in myocardial and liver ECV, 6 months after initiating chemotherapy, independently predict mortality (myocardial HR: 1.11 [95% CI: 1.02-1.20]; P = 0.011; liver HR: 1.07 [95% CI: 1.01-1.13]; P = 0.014). Conclusions: Multiorgan ECV quantification accurately tracks treatment response and demonstrates different rates of organ regression, with the liver and spleen regressing more rapidly than the heart. Baseline myocardial and liver ECV and changes at 6 months independently predict mortality, even after adjusting for traditional predictors of prognosis.
MeSH term(s)	Humans ; Contrast Media ; Predictive Value of Tests ; Immunoglobulin Light-chain Amyloidosis/pathology ; Amyloidosis/diagnostic imaging ; Amyloidosis/drug therapy ; Myocardium/pathology ; Amyloid ; Magnetic Resonance Spectroscopy ; Magnetic Resonance Imaging, Cine
Chemical Substances	Contrast Media ; Amyloid
Language	English
Publishing date	2023-05-10
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2491503-8
ISSN	1876-7591 ; 1936-878X
ISSN (online)	1876-7591
ISSN	1936-878X
DOI	10.1016/j.jcmg.2023.02.019
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Article ; Online: Prevalence, characteristics and outcomes of older patients with hereditary versus wild-type transthyretin amyloid cardiomyopathy.

European journal of heart failure

2023 Volume 25, Issue 4, Page(s) 515–524

Abstract: Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is often assumed to be associated with wild-type TTR genotype (ATTRwt) in elderly patients (aged ≥70), some of whom are not offered genetic testing. We sought to estimate the prevalence, clinical ... ...

Abstract	Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is often assumed to be associated with wild-type TTR genotype (ATTRwt) in elderly patients (aged ≥70), some of whom are not offered genetic testing. We sought to estimate the prevalence, clinical characteristics and prognostic implications of transthyretin (TTR) variants among elderly patients diagnosed with ATTR-CM. Methods and results: Data from consecutive patients over 70 years of age diagnosed with ATTR-CM at the UK National Amyloidosis Centre between January 2010 and August 2022 were retrospectively evaluated. All patients underwent clinical evaluation, biochemical tests, echocardiography and TTR genotyping. The study outcome was all-cause mortality. The study population consisted of 2029 patients with ATTR-CM (median age 79 years at diagnosis, 13.5% females, 80.4% Caucasian). Variant ATTR-CM (ATTRv-CM) was diagnosed in 20.7% (n = 421) of the study population of whom 327 (77.7%) carried V122I, 47 (11.2%) T60A, 16 (3.8%) V30M and 31 (7.3%) other pathogenic TTR variants. During a median (range) follow-up of 29 (12-48) months, ATTRv-CM was associated with increased all-cause mortality compared to ATTRwt-CM, with the poorest survival observed in V122I-associated ATTRv-CM (p < 0.001). Univariable and multivariable logistic regression analyses in those with ATTR-CM showed younger age at diagnosis (odds ratio [OR] 0.85 per year, p < 0.001), female sex (OR 2.73, p < 0.001), Afro-Caribbean ethnicity (OR 65.5, p < 0.001), atrial fibrillation (OR 0.65, p = 0.015), ischaemic heart disease (OR 0.54, p = 0.007), peripheral polyneuropathy (OR 5.70, p < 0.001) and orthostatic hypotension (OR 6.29, p < 0.001) to be independently associated with ATTRv-CM. Conclusion: Up to 20.7% of elderly patients with ATTR-CM have a pathogenic TTR variant. These findings support routine sequencing of the TTR gene in all patients with ATTR-CM regardless of age.
MeSH term(s)	Aged ; Humans ; Female ; Aged, 80 and over ; Male ; Prevalence ; Prealbumin/genetics ; Amyloid Neuropathies, Familial/epidemiology ; Amyloid Neuropathies, Familial/genetics ; Amyloid Neuropathies, Familial/complications ; Retrospective Studies ; Heart Failure/complications ; Cardiomyopathies/epidemiology ; Cardiomyopathies/genetics ; Cardiomyopathies/diagnosis
Chemical Substances	Prealbumin
Language	English
Publishing date	2023-01-30
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1483672-5
ISSN	1879-0844 ; 1388-9842
ISSN (online)	1879-0844
ISSN	1388-9842
DOI	10.1002/ejhf.2776
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Zs.A 5299: Show issues

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Article ; Online: Role of

Sachchithanantham, Sajitha / Hutt, David F / Quigley, Ann-Marie / Hawkins, Philip / Wechalekar, Ashutosh D

British journal of haematology

2017 Volume 183, Issue 3, Page(s) 506–509

MeSH term(s)	Aged ; Cardiomyopathies/diagnostic imaging ; Cardiomyopathies/metabolism ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis/diagnostic imaging ; Immunoglobulin Light-chain Amyloidosis/metabolism ; Male ; Middle Aged ; Radionuclide Imaging ; Technetium/administration & dosage
Chemical Substances	Technetium (7440-26-8)
Language	English
Publishing date	2017-10-30
Publishing country	England
Document type	Letter
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/bjh.14988
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Article ; Online: The Authors Reply.

Fontana, Marianna / Martinez-Naharro, Ana / Chacko, Liza / Rowczenio, Dorota / Gilbertson, Janet A / Whelan, Carol J / Strehina, Svetla / Lane, Thirusha / Moon, James C / Hutt, David F / Kellman, Peter / Petrie, Aviva / Hawkins, Philip N / Gillmore, Julian D

JACC. Cardiovascular imaging

2021 Volume 14, Issue 4, Page(s) 882–883

Language	English
Publishing date	2021-03-12
Publishing country	United States
Document type	Letter ; Comment
ZDB-ID	2491503-8
ISSN	1876-7591 ; 1936-878X
ISSN (online)	1876-7591
ISSN	1936-878X
DOI	10.1016/j.jcmg.2021.02.003
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Article ; Online: Comparison of different technetium-99m-labelled bone tracers for imaging cardiac amyloidosis.

Porcari, Aldostefano / Hutt, David F / Grigore, Simona F / Quigley, Ann-Marie / Rowczenio, Dorota / Gilbertson, Janet / Patel, Rishi / Razvi, Yousuf / Ioannou, Adam / Rauf, Muhammad U / Martinez-Naharro, Ana / Venneri, Lucia / Hawkins, Philip N / Fontana, Marianna / Gillmore, Julian D

European journal of preventive cardiology

2022 Volume 30, Issue 3, Page(s) e4–e6

MeSH term(s)	Humans ; Technetium ; Amyloidosis/diagnostic imaging ; Cardiomyopathies/diagnostic imaging ; Diagnostic Imaging
Chemical Substances	Technetium (7440-26-8)
Language	English
Publishing date	2022-10-17
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2626011-6
ISSN	2047-4881 ; 2047-4873
ISSN (online)	2047-4881
ISSN	2047-4873
DOI	10.1093/eurjpc/zwac237
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Zs.A 4686: Show issues

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Article ; Online: Clinical and Prognostic Implications of Right Ventricular Uptake on Bone Scintigraphy in Transthyretin Amyloid Cardiomyopathy.

Porcari, Aldostefano / Fontana, Marianna / Canepa, Marco / Biagini, Elena / Cappelli, Francesco / Gagliardi, Christian / Longhi, Simone / Pagura, Linda / Tini, Giacomo / Dore, Franca / Bonfiglioli, Rachele / Bauckneht, Matteo / Miceli, Alberto / Girardi, Francesca / Martini, Anna Lisa / Barbati, Giulia / Costanzo, Egidio Natalino / Caponetti, Angelo Giuseppe / Paccagnella, Andrea /

Sguazzotti, Maurizio / La Malfa, Giovanni / Zampieri, Mattia / Sciagrà, Roberto / Perfetto, Federico / Rowczenio, Dorota / Gilbertson, Janet / Hutt, David F / Hawkins, Philip N / Rapezzi, Claudio / Merlo, Marco / Sinagra, Gianfranco / Gillmore, Julian D

Circulation

2024 Volume 149, Issue 15, Page(s) 1157–1168

Abstract: Background: The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac amyloid burden ... ...

Abstract	Background: The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac amyloid burden and be associated with outcome. Methods: Consecutive patients with ATTR-CM who underwent diagnostic bone tracer scintigraphy with acquisition of whole-body planar and cardiac single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre and 4 Italian centers were included. Cardiac uptake was defined according to the Perugini classification: 0=absent cardiac uptake; 1=mild uptake less than bone; 2=moderate uptake equal to bone; and 3=high uptake greater than bone. Extent of right ventricular (RV) uptake was defined as focal (basal segment of the RV free wall only) or diffuse (extending beyond basal segment) on the basis of SPECT imaging. The primary outcome was all-cause mortality. Results: Among 1422 patients with ATTR-CM, RV uptake accompanying left ventricular uptake was identified by SPECT imaging in 100% of cases at diagnosis. Median follow-up in the whole cohort was 34 months (interquartile range, 21 to 50 months), and 494 patients died. By Kaplan-Meier analysis, diffuse RV uptake on SPECT imaging (n=936) was associated with higher all-cause mortality compared with focal (n=486) RV uptake (77.9% versus 22.1%; Conclusions: Diffuse RV uptake of bone tracer on SPECT imaging is associated with poor outcomes in patients with ATTR-CM and is an independent prognostic marker at diagnosis.
MeSH term(s)	Humans ; Cardiomyopathies/diagnosis ; Prealbumin/genetics ; Prognosis ; Tomography, Emission-Computed, Single-Photon
Chemical Substances	Prealbumin
Language	English
Publishing date	2024-02-08
Publishing country	United States
Document type	Journal Article
ZDB-ID	80099-5
ISSN	1524-4539 ; 0009-7322 ; 0069-4193 ; 0065-8499
ISSN (online)	1524-4539
ISSN	0009-7322 ; 0069-4193 ; 0065-8499
DOI	10.1161/CIRCULATIONAHA.123.066524
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Article ; Online: The role of serial

Ross, James C / Hutt, David F / Burniston, Maria / Grigore, Simona F / Fontana, Marianna / Page, Joanne / Hawkins, Philip N / Gilbertson, Janet A / Rowczenio, Dorota / Gillmore, Julian D

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

2021 Volume 29, Issue 1, Page(s) 38–49

Abstract: Purpose: Cardiac transthyretin amyloidosis is a usually fatal form of restrictive cardiomyopathy for which clinical trials of treatments are ongoing. It is anticipated that quantitative nuclear medicine scintigraphy, which is experiencing growing ... ...

Abstract	Purpose: Cardiac transthyretin amyloidosis is a usually fatal form of restrictive cardiomyopathy for which clinical trials of treatments are ongoing. It is anticipated that quantitative nuclear medicine scintigraphy, which is experiencing growing interest, will soon be used to evaluate treatment efficacy. We investigated its utility for monitoring changes in disease load over a significant time period. Methods: Sixty-two treatment-naive patients underwent Results: In total 170 datasets were analysed. Increased myocardial retention of Conclusions: The spread of our results was notably high compared to the year-on-year increases. If left unaccounted for, variance may draw fallacious conclusions about changes in disease load. We therefore urge caution in drawing conclusions solely from nuclear medicine scintigraphy on a patient-by-patient basis, particularly across a short time period.
MeSH term(s)	Amyloid Neuropathies, Familial/diagnostic imaging ; Cardiomyopathies/diagnostic imaging ; Diphosphonates ; Humans ; Organotechnetium Compounds ; Radionuclide Imaging ; Tomography, X-Ray Computed
Chemical Substances	Diphosphonates ; Organotechnetium Compounds
Language	English
Publishing date	2021-10-27
Publishing country	England
Document type	Journal Article
ZDB-ID	1205246-2
ISSN	1744-2818 ; 1350-6129
ISSN (online)	1744-2818
ISSN	1350-6129
DOI	10.1080/13506129.2021.1991302
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