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Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping

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...

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Autores principales: Ioannou, Adam, Patel, Rishi K., Martinez-Naharro, Ana, Razvi, Yousuf, Porcari, Aldostefano, Hutt, David F., Bandera, Francesco, Kotecha, Tushar, Venneri, Lucia, Chacko, Liza, Massa, Paolo, Hanger, Melissa, Knight, Daniel, Manisty, Charlotte, Moon, James, Quarta, Cristina, Lachmann, Helen, Whelan, Carol, Kellman, Peter, Hawkins, Philip N., Gillmore, Julian D., Wechelakar, Ashutosh, Fontana, Marianna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10406611/
https://www.ncbi.nlm.nih.gov/pubmed/37178079
http://dx.doi.org/10.1016/j.jcmg.2023.02.019
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author Ioannou, Adam
Patel, Rishi K.
Martinez-Naharro, Ana
Razvi, Yousuf
Porcari, Aldostefano
Hutt, David F.
Bandera, Francesco
Kotecha, Tushar
Venneri, Lucia
Chacko, Liza
Massa, Paolo
Hanger, Melissa
Knight, Daniel
Manisty, Charlotte
Moon, James
Quarta, Cristina
Lachmann, Helen
Whelan, Carol
Kellman, Peter
Hawkins, Philip N.
Gillmore, Julian D.
Wechelakar, Ashutosh
Fontana, Marianna
author_facet Ioannou, Adam
Patel, Rishi K.
Martinez-Naharro, Ana
Razvi, Yousuf
Porcari, Aldostefano
Hutt, David F.
Bandera, Francesco
Kotecha, Tushar
Venneri, Lucia
Chacko, Liza
Massa, Paolo
Hanger, Melissa
Knight, Daniel
Manisty, Charlotte
Moon, James
Quarta, Cristina
Lachmann, Helen
Whelan, Carol
Kellman, Peter
Hawkins, Philip N.
Gillmore, Julian D.
Wechelakar, Ashutosh
Fontana, Marianna
author_sort Ioannou, Adam
collection PubMed
description 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.
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spelling pubmed-104066112023-08-09 Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping Ioannou, Adam Patel, Rishi K. Martinez-Naharro, Ana Razvi, Yousuf Porcari, Aldostefano Hutt, David F. Bandera, Francesco Kotecha, Tushar Venneri, Lucia Chacko, Liza Massa, Paolo Hanger, Melissa Knight, Daniel Manisty, Charlotte Moon, James Quarta, Cristina Lachmann, Helen Whelan, Carol Kellman, Peter Hawkins, Philip N. Gillmore, Julian D. Wechelakar, Ashutosh Fontana, Marianna JACC Cardiovasc Imaging Original Research 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. Elsevier 2023-08 /pmc/articles/PMC10406611/ /pubmed/37178079 http://dx.doi.org/10.1016/j.jcmg.2023.02.019 Text en © 2023 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Original Research
Ioannou, Adam
Patel, Rishi K.
Martinez-Naharro, Ana
Razvi, Yousuf
Porcari, Aldostefano
Hutt, David F.
Bandera, Francesco
Kotecha, Tushar
Venneri, Lucia
Chacko, Liza
Massa, Paolo
Hanger, Melissa
Knight, Daniel
Manisty, Charlotte
Moon, James
Quarta, Cristina
Lachmann, Helen
Whelan, Carol
Kellman, Peter
Hawkins, Philip N.
Gillmore, Julian D.
Wechelakar, Ashutosh
Fontana, Marianna
Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
title Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
title_full Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
title_fullStr Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
title_full_unstemmed Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
title_short Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
title_sort tracking multiorgan treatment response in systemic al-amyloidosis with cardiac magnetic resonance derived extracellular volume mapping
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10406611/
https://www.ncbi.nlm.nih.gov/pubmed/37178079
http://dx.doi.org/10.1016/j.jcmg.2023.02.019
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