ArticleJACC. Cardiovascular imaging2023
Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping.
Article in JACC. Cardiovascular imaging, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 34 citations in OpenAlex.
- Prognostic value of CMR parametric mapping in cardiac amyloidosis: an updated systematic review and meta-analysis.Open heart · 2025Pooled it
- Beyond the Heart: Hepatic Extracellular Volume Assessment in Patients With Cardiovascular Disease.Journal of magnetic resonance imaging : JMRI · 2026Article
- Imaging Advances in Light Chain Amyloidosis.Diagnostics (Basel, Switzerland) · 2026Review
- Multimodal Imaging for Monitoring of Disease Progression in Cardiac Amyloidosis: Advances and Gaps in Evidence.Journal of cardiovascular development and disease · 2026Review
- Transthyretin amyloid cardiomyopathy: from cause to novel treatments.European heart journal · 2026Review
- Promises and challenges of AI-enabled methods for myocardial characterisation in cardiovascular magnetic resonance.Frontiers in cardiovascular medicine · 2026Review
- Transthyretin amyloid cardiomyopathy: natural history and treatment response assessed by cardiovascular magnetic resonance.European heart journal · 2025Article
- Article
- Microvascular obstruction in cardiac amyloidosis.European journal of heart failure · 2025Article
- Non-Invasive Diagnostic Algorithm in Transthyretin Cardiac Amyloidosis: Is Bone Scintigraphy Always Enough?Journal of clinical medicine · 2025Review
- Correlation between the extracellular volume fraction and hemodynamic parameters in light-chain cardiac amyloidosis patients.Quantitative imaging in medicine and surgery · 2025Article
- The antibody-peptide fusion protein, AT-02, is an effective opsonin with pan-amyloid reactivity.npj drug discovery · 2025Article
- Recent developments in systemic light-chain amyloidosis prognosis and treatment.Future cardiology · 2025Review
- Development of Imaging Endpoints for Clinical Trials in AL and ATTR Amyloidosis: Proceedings of the Amyloidosis Forum.JACC. Cardiovascular imaging · 2025Review
- The Last Decade in Cardiac Amyloidosis: Advances in Understanding Pathophysiology, Diagnosis and Quantification, Prognosis, Treatment Strategies, and Monitoring Response.JACC. Cardiovascular imaging · 2025Review
- Monitoring Disease Progression in Transthyretin Amyloid Cardiomyopathy.Heart international · 2025Review
- Stratifying Disease Progression in Patients With Cardiac ATTR Amyloidosis.Journal of the American College of Cardiology · 2024Article
- Evolution of Disease-modifying Therapy for Transthyretin Cardiac Amyloidosis.Heart international · 2024Review
- Complex Connections: Myocardial Fibrosis, Reproductive Factors, and Heart Failure.JACC. Advances · 2023Article
- The role of imaging in the selection of patients for HFpEF therapy.European heart journal. Cardiovascular Imaging · 2023Review
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23 authors at 6 institutions in 3 countries.
Funding
Abstract
backgroundSystemic 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.
objectivesThe purpose of this study was to assess multiorgan response to treatment using ECV mapping, and assess the association between multiorgan treatment response and prognosis.
methodsThe 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.
resultsAt 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).
conclusionsMultiorgan 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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