• Eur. J. Intern. Med. · Dec 2020

    Review

    Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery.

    • Aldostefano Porcari, Marco Merlo, Claudio Rapezzi, and Gianfranco Sinagra.
    • Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Italy.
    • Eur. J. Intern. Med. 2020 Dec 1; 82: 7-15.

    AbstractTransthyretin amyloid cardiomyopathy (ATTR-AC) is an under-recognized and underdiagnosed disease. Although traditionally considered a rare condition, the epidemiology of the disease is rapidly changing due to the possibility of non-invasive diagnosis through cardiac scintigraphy with bone tracers and novel disease-modifying treatments providing survival advantages. Nevertheless, many questions and grey areas have to be addressed, such as the natural history of ATTR-AC, the role and implications of genotype-phenotype interactions, the best clinical management, prognostic stratification and the most appropriate treatments, including those already recommended for patients with heart failure. Clinicians have to cope with old beliefs and evolving concepts in ATTR-AC. A wide horizon of possibilities for physicians of many specialties is unfolding and awaits discovery.Copyright © 2020. Published by Elsevier B.V.

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