Patel, Rishi; (2025) Multimodal imaging in the assessment of disease phenotypes, progression, and prognosis of Cardiac Amyloidosis. Doctoral thesis (Ph.D), UCL (University College London).

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Text Rishi Patel - PhD thesis - UCL (University College London) - Multimodal imaging in cardiac amyloidosis.pdf - Accepted Version Download (6MB) | Preview

Abstract

Background: Systemic amyloidoses are characterised by deposition of misfolded proteins within the extracellular space of multiple organs. Cardiac amyloid deposition results in a progressive decline in cardiac function, manifesting clinically as heart failure, and is the major course of mortality. / Aims: To use non-invasive techniques to improve our understanding of the amyloidoses from diagnosis, through disease progression and prognosis: (1) characterise sex differences in Transthyretin amyloid cardiomyopathy (ATTR-CM) using echocardiography, (2) measure the ability of cardiovascular magnetic resonance (CMR) to assess disease progression in ATTR-CM and to assess treatment response and prognosis in both ATTR-CM and systemic light chain (AL) amyloidosis, and (3) to assess functional phenotypes, treatment response and prognosis of ATTR-CM using cardiopulmonary exercise testing (CPET). / Results and Conclusions: We found no differences in the clinical phenotype between males and females with ATTR-CM. Current guidelines may inaccurately perceive women to have a milder clinical phenotype, when in fact they present at an older age and, as a result, with more advance disease. Using CMR, we found that extracellular volume (ECV) mapping can track changes in cardiac amyloid load over time in both AL and ATTR-CM. In both subtypes, treatment response was variable but correlated with structural and functional changes, and an increase in ECV of >5% was independently predictive of mortality. Using CPET, functional impairment was highly prevalent in ATTR disease, peak VO2 and blood pressure were independent predictors of prognosis and disease-modifying treatment resulted in significant preservation in inotropic reserve and relative improvement in ventilatory efficiency. / Conclusions: The management of cardiac amyloidosis requires a multidimensional approach, where the applications of echocardiography, CMR and CPET at different times in the patient journey can not only improve diagnostics, disease monitoring and risk stratification, but may be of great value for early-stage drug development in clinical trials.

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