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Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

Martinez de Azcona Naharro, Ana; (2020) Cardiovascular Magnetic Resonance in Cardiac Amyloidosis. Doctoral thesis (Ph.D), UCL (University College London). Green open access

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Abstract

Background: Systemic amyloidoses are an underdiagnosed, but increasingly recognized group of progressive disorders characterised by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative or restrictive cardiomyopathy and is the major driver of prognosis. / Aims: In this thesis, by using cardiovascular magnetic resonance (CMR), I have aimed to assess the cardiac response to chemotherapy in AL amyloidosis; to assess morphological phenotypes and tissue characterization findings in ATTR cardiac amyloidosis, and compare these findings with AL amyloidosis; to evaluate the prognostic potential of native myocardial T1 in ATTR cardiac amyloidosis and compare native T1 with extracellular volume (ECV) in terms of diagnostic accuracy and prognosis; to study the prevalence of thrombus in the left atrial appendage in the cardiac amyloidosis population; and to explore the role of hypoperfusion at rest in cardiac amyloidosis. / Results and Conclusions: I confirmed that CMR with T1 mapping and ECV measurements demonstrates that cardiac AL amyloid deposits frequently regress following chemotherapy that substantially suppresses clonal light chain production. I characterised the cardiac morphology in ATTR cardiac amyloidosis. I demonstrated that native T1 mapping and ECV are good diagnostic techniques in cardiac ATTR amyloidosis that associate with prognosis. Both parameters also correlate with mortality, but only ECV remains independently predictive of prognosis. I confirmed that the prevalence of intracardiac thrombi in cardiac amyloidosis and atrial fibrillation is high despite anticoagulation, with significant thrombus prevalence even in sinus rhythm. I demonstrated that myocardial hypoperfusion is common and substantial in cardiac amyloidosis. CMR indicates a complex pathophysiology in which systolic dysfunction, diastolic dysfunction, and amyloid deposition are independently associated with reduced myocardial perfusion. Patients with amyloidosis continue to have unmet needs, many of which stem from heart involvement, but outcomes are gradually improving.

Type: Thesis (Doctoral)
Qualification: Ph.D
Title: Cardiovascular Magnetic Resonance in Cardiac Amyloidosis
Event: UCL (University College London)
Open access status: An open access version is available from UCL Discovery
Language: English
Additional information: Copyright © The Author 2020. Original content in this thesis is licensed under the terms of the Creative Commons Attribution 4.0 International (CC BY 4.0) Licence (https://creativecommons.org/licenses/by/4.0/). Any third-party copyright material present remains the property of its respective owner(s) and is licensed under its existing terms. Access may initially be restricted at the author’s request.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
URI: https://discovery.ucl.ac.uk/id/eprint/10116540
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