Use this URL to cite or link to this record in EThOS: https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.822768
Title: Cardiovascular magnetic resonance in cardiac amyloidosis
Author: Martinez de Azcona Naharro, Ana
ISNI:       0000 0005 0288 5364
Awarding Body: UCL (University College London)
Current Institution: University College London (University of London)
Date of Award: 2020
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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.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID: uk.bl.ethos.822768  DOI: Not available
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