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Myocardial perfusion in cardiac amyloidosis

Title: Myocardial perfusion in cardiac amyloidosis
Authors: Chacko, Liza; Kotecha, Tushar; Ioannou, Adam; Patel, Niket; Martinez‐Naharro, Ana; Razvi, Yousuf; Patel, Rishi; Massa, Paolo; Venneri, Lucia; Brown, James; Porcari, Aldostefano; Knott, Kristopher; Manisty, Charlotte; Knight, Daniel; Lockie, Tim; Rakhit, Roby; Lachmann, Helen; Wechelakar, Ashutosh; Whelan, Carol; Ponticos, Markella; Moon, James; González, Arantxa; Gilbertson, Janet; Riefolo, Mattia; Leone, Ornella; Xue, Hui; Hawkins, Philip; Kellman, Peter; Gillmore, Julian; Fontana, Marianna
Contributors: Chacko, Liza; Kotecha, Tushar; Ioannou, Adam; Patel, Niket; Martinez‐naharro, Ana; Razvi, Yousuf; Patel, Rishi; Massa, Paolo; Venneri, Lucia; Brown, Jame; Porcari, Aldostefano; Knott, Kristopher; Manisty, Charlotte; Knight, Daniel; Lockie, Tim; Rakhit, Roby; Lachmann, Helen; Wechelakar, Ashutosh; Whelan, Carol; Ponticos, Markella; Moon, Jame; González, Arantxa; Gilbertson, Janet; Riefolo, Mattia; Leone, Ornella; Xue, Hui; Hawkins, Philip; Kellman, Peter; Gillmore, Julian; Fontana, Marianna
Publication Year: 2024
Collection: Università degli studi di Trieste: ArTS (Archivio della ricerca di Trieste)
Subject Terms: Amyloidosi; Extracellular volume; Infiltration; Ischaemia; Magnetic resonance imaging; Perfusion
Description: Aims: Cardiac involvement is the main driver of clinical outcomes in systemic amyloidosis and preliminary studies support the hypothesis that myocardial ischaemia contributes to cellular damage. The aims of this study were to assess the presence and mechanisms of myocardial ischaemia using cardiovascular magnetic resonance (CMR) with multiparametric mapping and histopathological assessment. Methods and results: Ninety-three patients with cardiac amyloidosis (CA) (light-chain amyloidosis n = 42, transthyretin amyloidosis n = 51) and 97 without CA (three-vessel coronary disease [3VD] n = 47, unobstructed coronary arteries n = 26, healthy volunteers [HV] n = 24) underwent quantitative stress perfusion CMR with myocardial blood flow (MBF) mapping. Twenty-four myocardial biopsies and three explanted hearts with CA were analysed histopathologically. Stress MBF was severely reduced in patients with CA with lower values than patients with 3VD, unobstructed coronary arteries and HV (CA: 1.04 ± 0.51 ml/min/g, 3VD: 1.35 ± 0.50 ml/min/g, unobstructed coronary arteries: 2.92 ± 0.52 ml/min/g, HV: 2.91 ± 0.73 ml/min/g; CA vs. 3VD p = 0.011, CA vs. unobstructed coronary arteries p < 0.001, CA vs. HV p < 0.001). Myocardial perfusion abnormalities correlated with amyloid burden, systolic and diastolic function, structural parameters and blood biomarkers (p < 0.05). Biopsies demonstrated abnormal vascular endothelial growth factor staining in cardiomyocytes and endothelial cells, which may be related to hypoxia conditions. Amyloid infiltration in intramural arteries was associated with severe lumen reduction and severe reduction in capillary density. Conclusion: Cardiac amyloidosis is associated with severe inducible myocardial ischaemia demonstrable by histology and CMR stress perfusion mapping. Histological evaluation indicates a complex pathophysiology, where in addition to systolic and diastolic dysfunction, amyloid infiltration of the epicardial arteries and disruption and rarefaction of the capillaries play a role ...
Document Type: article in journal/newspaper
Language: English
Relation: info:eu-repo/semantics/altIdentifier/pmid/38247182; info:eu-repo/semantics/altIdentifier/wos/WOS:001146161300001; volume:26; issue:3; firstpage:598; lastpage:609; numberofpages:12; journal:EUROPEAN JOURNAL OF HEART FAILURE; https://hdl.handle.net/11368/3069196
DOI: 10.1002/ejhf.3137
Availability: https://hdl.handle.net/11368/3069196; https://doi.org/10.1002/ejhf.3137; https://onlinelibrary.wiley.com/doi/10.1002/ejhf.3137
Rights: info:eu-repo/semantics/openAccess
Accession Number: edsbas.B4739EEB
Database: BASE