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<https://discovery.ucl.ac.uk/id/eprint/10197505> <http://purl.org/dc/terms/title> "Cardiac amyloidosis: The journey from diagnosis to treatment"^^<http://www.w3.org/2001/XMLSchema#string> .
<https://discovery.ucl.ac.uk/id/eprint/10197505> <http://purl.org/ontology/bibo/abstract> "Background\r\nThe systemic amyloidoses are a heterogenous group of diseases characterised by deposition of\r\namyloid fibrils within the extracellular space of various organs. Accumulation within the\r\nmyocardium results in an infiltrative cardiomyopathy, and is the main driver of prognosis.\r\nAims\r\nThis thesis aims to: (1) characterise different forms of cardiac amyloidosis (CA) though use of\r\nmultimodality cardiac imaging; (2) characterise the impact of earlier diagnosis in patients with\r\ntransthyretin cardiac amyloidosis (ATTR-CA); (3) identify novel prognostic biomarkers for\r\npatients with ATTR-CA; (4) evaluate the use of heart failure therapies in ATTR-CA; (5) identify\r\nnovel markers of disease progression in ATTR-CA; (6) identify novel imaging-based markers\r\nthat track the treatment response in cardiac AL amyloidosis.\r\nResults and conclusions\r\nThis thesis demonstrated that a combination of advanced cardiac imaging can refine the\r\ndifferentials in suspected CA. Multimodality imaging can characterise rare forms of CA, and\r\nhas identified clinical features that should prompt the consideration of rarer forms within the\r\ndifferentials. ATTR-CA patients are being diagnosed earlier in the course of their disease, with\r\na milder cardiac phenotype at diagnosis and reduced mortality from the time of diagnosis,\r\nwhich ought to be factored into the design of clinical trials. Various novel biomarkers have\r\nbeen identified as being associated with mortality in ATTR-CA, and can refine risk\r\nstratification beyond the conventional staging system. Mineralocorticoid receptor antagonists,\r\nand beta-blockers in patients with a left ventricular ejection fraction <40% were associated\r\n4\r\nwith a reduced risk of mortality, and offer new treatment options for ATTR-CA. This thesis\r\nidentified novel definitions of NT-proBNP progression, and 6-minute walk test distance\r\nworsening, which combined with outpatient diuretic intensification detect disease progression\r\nin ATTR-CA. Cardiac magnetic resonance imaging can track the treatment response in cardiac\r\nAL amyloidosis, and guide whether treatment can be safely discontinued or if treatment\r\nintensification is required."^^<http://www.w3.org/2001/XMLSchema#string> .
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