Novel insights in cardiac imaging to evaluate disease progression and mechanisms in cardiac amyloidosis

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Novel insights in cardiac imaging to evaluate disease progression and mechanisms in cardiac amyloidosis

Chacko, Liza; (2023) Novel insights in cardiac imaging to evaluate disease progression and mechanisms in cardiac amyloidosis. Doctoral thesis (Ph.D), UCL (University College London). Green open access

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Abstract

Introduction: Systemic amyloidosis is characterised by the extracellular deposition of amyloid fibrils that misfold and aggregate causing disease when accumulation is sufficient to disrupt the structure and integrity of affected organs. Cardiac involvement causes a restrictive cardiomyopathy and determines prognosis. / Aims: My aims were 1) To assess the echocardiographic phenotype of patients with transthyretin (ATTR) cardiomyopathy and evaluate determinants of prognosis at baseline 2) To describe the echocardiographic progression in patients with ATTR cardiomyopathy over serial assessment 3) To assess the role of cardiac magnetic resonance (CMR)-derived extracellular volume (ECV) mapping to detect and estimate extra-cardiac amyloid load against Serum Amyloid P (SAP) scintigraphy 4) To assess the presence and mechanisms of myocardial ischaemia using stress perfusion CMR. / Results and Conclusions: Results demonstrate that 1) At baseline echocardiographic assessment in ATTR cardiomyopathy, stroke volume index, right atrial area index, longitudinal strain, E/e’ and severe aortic stenosis were independently associated with mortality. Compared to other genotypes, patients with V122I-associated ATTR cardiomyopathy had the most severe degree of dysfunction. 2) Worsening in the degree of mitral and tricuspid regurgitation at 12 and-24 months was associated with worse prognosis by echocardiography, and patients with V122-associated ATTR cardiomyopathy had more rapid progression when compared to other genotypes. 3) CMR derived ECV mapping shows high diagnostic performance to detect splenic and hepatic amyloid deposits with good correlation between ECV and amyloid load assessed by SAP scintigraphy. 4) Myocardial blood flow assessed by stress perfusion CMR is severely reduced in patients with cardiac amyloidosis and histological analysis of endomyocardial tissue shows evidence of hypoxia, vascular infiltration and capillary rarefaction. In conclusion, these results demonstrate that imaging in cardiac amyloidosis has an important role in optimising diagnosis, tracking individual changes over time and characterising amyloid organ involvement.

Type:	Thesis (Doctoral)
Qualification:	Ph.D
Title:	Novel insights in cardiac imaging to evaluate disease progression and mechanisms in cardiac amyloidosis
Open access status:	An open access version is available from UCL Discovery
Language:	English
Additional information:	Copyright © The Author 2023. 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/10174283