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Revista chilena de cardiología

On-line version ISSN 0718-8560

Abstract

CASTANIA, Felipe et al. Transthyretin amyloid cardiomyopathy: a review. Rev Chil Cardiol [online]. 2021, vol.40, n.2, pp.148-160. ISSN 0718-8560.  http://dx.doi.org/10.4067/S0718-85602021000200148.

Transthyretin amyloid cardiomyopathy (AT-TR-CM) is a disease characterized by extracellular deposition of amyloid fibrils in the myocardium, from misfolded transthyretin, generating a restrictive cardiomyopathy. This misfolded protein may be inherited or acquired, and is more prevalent in elderly patients. ATTR-CM has emerged as an underdiagnosed cause of heart failure with preserved ejection fraction (HF-PEF). The fundamental pillarfor its diagnosis is high clinical suspicion since the symptoms are usually nonspecific. The diagnosis can be made from bone scintigraphy, reserving myocardial biopsy for particular situations. With the advent of new therapies that affect the survival of these patients, a timely diagnosis has become crucial.

Keywords : cardiac amyloidosis; transthyretin amyloid cardiomyopathy.

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