Abstract
Cardiac amyloidosis (CA) is a progressive and often fatal disorder that may be greatly underdiagnosed and is certainly an underappreciated cause of heart failure. More than 30 proteins can form amyloid fibrils, and the difference in the precursor protein forms the basis for the classification. Almost all clinical cases of CA are from either misfolded monoclonal immunoglobulin light chains (AL or primary systemic) from an abnormal clonal proliferation of plasma cells, or transthyretin amyloidosis (ATTR), a liver-synthesized protein normally involved in the transportation of the hormone thyroxine and retinol-binding protein. The use of cardiac magnetic resonance (CMR) imaging and bone scintigraphy has revolutionized the diagnostic approach to CA, reducing the need for histological evaluation by endomyocardial biopsy. CMR imaging might raise the suspicion of CA and differentiate it from other cardiomyopathies with hypertrophic phenotype. Furthermore, if noninvasive criteria for the diagnosis of CA are not satisfied, CMR findings might strengthen the decision to pursue histological evidence of amyloid deposition in the heart. Following the development of disease-modifying drugs with a proven impact on patients’ survival, the hardest clinical challenge is the recognition of CA at early stages. CMR is a paramount imaging technique in the early detection of CA, when the highest benefits are expected from therapy and is important in the assessment of treatment response, guiding clinicians on when to escalate the dose, switch or combine different therapies, and when to stop treatment in non-responders. Late gadolinium enhancement, T1 values and extracellular volume fraction measured by CMR provide an accurate assessment of cardiac amyloid deposition, from initial to late stages of disease, with crucial diagnostic and prognostic information. Current management of patients with suspected and confirmed CA should rely on a multiparametric approach including a combination of echocardiography, CMR, and nuclear imaging integrated in routine clinical practice.
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Porcari, A., Sinagra, G., Fontana, M., Pica, S. (2023). Cardiac Amyloidosis. In: Barison, A., Dellegrottaglie, S., Pontone, G., Indolfi, C. (eds) Case-based Atlas of Cardiovascular Magnetic Resonance. Springer, Cham. https://doi.org/10.1007/978-3-031-32593-9_7
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