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The Echocardiogram in Amyloidosis

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Amyloidosis and Fabry Disease

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Abstract

Amyloidosis is a systemic disease caused by the extracellular deposition of insoluble amyloid fibrils in the heart and the echocardiogram is a key diagnostic method for the suspicion and diagnosis of this and other cardiac infiltrative diseases. Most of the classic and more specific findings of the disease are not present until later stages of infiltrative burden; in the early stages, it lacks specificity to precisely distinguish amyloid from nonamyloid infiltrative or hypertrophic heart diseases. The classical findings are biatrial enlargement, valvular and interatrial septum thickening, pleural and pericardial effusion, and biventricular hypertrophy with a bright and sparkling appearance with preserved left ventricular ejection fraction associated with diastolic dysfunction with a restrictive pattern.

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Authors and Affiliations

  1. Department of Internal Medicine at the Federal University of Paraíba, João Pessoa, PB, Brazil

    Marcelo Dantas Tavares de Melo

  2. Clinical Medicine of State of Paraiba, João Pessoa, PB, Brazil

    Marcelo Dantas Tavares de Melo

  3. Cardiology at Federal University of Paraiba, João Pessoa, PB, Brazil

    Marcelo Dantas Tavares de Melo

  4. Cardiovascular Imaging Department, Brazilian Society of Cardiology, Rio de Janeiro, RJ, Brazil

    Alex dos Santos Félix

  5. National Institute of Cardiology, Rio de Janeiro, RJ, Brazil

    Alex dos Santos Félix

  6. Cardiology—DASA, Rio de Janeiro, RJ, Brazil

    Alex dos Santos Félix

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  1. Marcelo Dantas Tavares de Melo
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Correspondence to Marcelo Dantas Tavares de Melo .

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Editors and Affiliations

  1. Postgraduate Program in Cardiovascular Science, Fluminense Federal University, Niteroi, Rio de Janeiro, Brazil

    Diane Xavier de Ávila

  2. Cardiology Division, Fluminense Federal University, Niterói, Rio de Janeiro, Brazil

    Humberto Villacorta Junior

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de Melo, M.D.T., dos Santos Félix, A. (2023). The Echocardiogram in Amyloidosis. In: Xavier de Ávila, D., Villacorta Junior, H. (eds) Amyloidosis and Fabry Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-17759-0_16

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