Ioannou, Adam George Andrew;
(2024)
Cardiac amyloidosis: The journey from diagnosis to treatment.
Doctoral thesis (Ph.D), UCL (University College London).
Preview |
Text
PhD thesis Student Number 22026549.pdf - Accepted Version Download (17MB) | Preview |
Abstract
Background The systemic amyloidoses are a heterogenous group of diseases characterised by deposition of amyloid fibrils within the extracellular space of various organs. Accumulation within the myocardium results in an infiltrative cardiomyopathy, and is the main driver of prognosis. Aims This thesis aims to: (1) characterise different forms of cardiac amyloidosis (CA) though use of multimodality cardiac imaging; (2) characterise the impact of earlier diagnosis in patients with transthyretin cardiac amyloidosis (ATTR-CA); (3) identify novel prognostic biomarkers for patients with ATTR-CA; (4) evaluate the use of heart failure therapies in ATTR-CA; (5) identify novel markers of disease progression in ATTR-CA; (6) identify novel imaging-based markers that track the treatment response in cardiac AL amyloidosis. Results and conclusions This thesis demonstrated that a combination of advanced cardiac imaging can refine the differentials in suspected CA. Multimodality imaging can characterise rare forms of CA, and has identified clinical features that should prompt the consideration of rarer forms within the differentials. ATTR-CA patients are being diagnosed earlier in the course of their disease, with a milder cardiac phenotype at diagnosis and reduced mortality from the time of diagnosis, which ought to be factored into the design of clinical trials. Various novel biomarkers have been identified as being associated with mortality in ATTR-CA, and can refine risk stratification beyond the conventional staging system. Mineralocorticoid receptor antagonists, and beta-blockers in patients with a left ventricular ejection fraction <40% were associated 4 with a reduced risk of mortality, and offer new treatment options for ATTR-CA. This thesis identified novel definitions of NT-proBNP progression, and 6-minute walk test distance worsening, which combined with outpatient diuretic intensification detect disease progression in ATTR-CA. Cardiac magnetic resonance imaging can track the treatment response in cardiac AL amyloidosis, and guide whether treatment can be safely discontinued or if treatment intensification is required.
| Type: | Thesis (Doctoral) |
|---|---|
| Qualification: | Ph.D |
| Title: | Cardiac amyloidosis: The journey from diagnosis to treatment |
| Open access status: | An open access version is available from UCL Discovery |
| Language: | English |
| Additional information: | Copyright © The Author 2022. Original content in this thesis is licensed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0) Licence (https://creativecommons.org/licenses/by-nc/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 |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10197505 |
Loading...
Loading...Loading...Loading...Archive Staff Only
 |
View Item |
