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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...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2023
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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. |
format | Online Article Text |
id | pubmed-10406611 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
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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