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Transthyretin Amyloid Cardiomyopathy: Current Diagnostic Approach and Risk Stratification with Multimodality Imaging
Georgios Tziomalos1, Thomas Zegkos1, Eleftheria Baltagianni1
1Department of Cardiology, AHEPA Hospital, School of Medicine, Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece.
Abstract:
Amyloidosis is an infiltrative disease that may cause cardiomyopathy if the precursor protein that misfolds and forms the amyloid is transthyretic or plasma abnormal light chains. Transthyretin amyloid cardiomyopathy has to be diagnosed timely and accurately since there are specific treatment options to support the patients. Multimodality imaging including electrocardiography, echocardiography with strain imaging and cardiac magnetic resonance applying late gadolinium enhancement imaging, native T1 mapping and extracellular volume, raise a high suspicion of the disease and bone scintigraphy set the diagnosis even without the need of biopsy. However, the morbidity and mortality remain high and the need for risk stratification and assessment of the response to treatment are of paramount importance. Cardiac imaging biomarkers offer a thoughtful insight into the prognosis of these patients at diagnosis and after treatment. The current narrative review aims to enlighten the use of multimodality cardiac imaging in transthyretic amyloid cardiomyopathy throughout the disease pathogenesis and evolution from diagnosis to prognosis and response to treatment in a personalized manner.
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