Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
Aldostefano Porcari, Marco Merlo, Claudio Rapezzi, Gianfranco Sinagra, Aldostefano Porcari, Marco Merlo, Claudio Rapezzi, Gianfranco Sinagra
Abstract
Transthyretin 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.
Keywords: Cardiac amyloidosis; Disease-modifying therapies; Grey zones; Prognostic stratification; Transthyretin.
Conflict of interest statement
The authors declare they have no conflict of interest.
Copyright © 2020. Published by Elsevier B.V.
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References
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Source: PubMed