Transthyretin amyloid cardiomyopathy
Med Clin (Barc). 2021 Feb 12;156(3):126-134.
doi: 10.1016/j.medcli.2020.06.064.
Epub 2020 Oct 31.
[Article in
English,
Spanish]
Affiliations
- 1 Unidad de Insuficiencia Cardíaca y Cardiopatías Familiares, Servicio de Cardiología, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, España; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, España; Universidad Francisco de Vitoria (UFV), Pozuelo de Alarcón, España. Electronic address: pablogpavia@yahoo.es.
- 2 Unidad de Insuficiencia Cardíaca y Cardiopatías Familiares, Servicio de Cardiología, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, España; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, España.
Abstract
Transthyretin (TTR) cardiac amyloidosis is a severe, progressive, infiltrative disease caused by the deposition of TTR at cardiac level. It may be due to a genetic alteration in its hereditary form (ATTRv) or as a consequence of an age-related degenerative process (ATTRwt). Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that ATTR is more frequent than traditionally considered and that it is particularly relevant in patients over 65 years with heart failure or with aortic stenosis. With the appearance of several treatment options capable of modifying the natural history of ATTR, it is necessary for clinicians to be familiar with the diagnostic process and treatment of this disease. This review will cover the clinical spectrum of presentation of ATTR, its diagnosis and treatment.
Keywords:
Amiloidosis; Amiloidosis cardíaca; Amyloidosis; Cardiac amyloidosis; Heart failure; Insuficiencia cardíaca; Transthyretin; Transtiretina; Tratamiento; Treatment.
Copyright © 2020 Elsevier España, S.L.U. All rights reserved.
MeSH terms
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Amyloid Neuropathies, Familial* / diagnosis
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Amyloid Neuropathies, Familial* / genetics
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Amyloid Neuropathies, Familial* / therapy
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Cardiomyopathies* / diagnosis
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Cardiomyopathies* / genetics
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Heart
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Heart Failure* / diagnosis
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Heart Failure* / etiology
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Humans
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Prealbumin / genetics