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Inicio Medicina Clínica (English Edition) Transthyretin amyloid cardiomyopathy
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Vol. 156. Issue 3.
Pages 126-134 (February 2021)
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Vol. 156. Issue 3.
Pages 126-134 (February 2021)
Review
Transthyretin amyloid cardiomyopathy
Amiloidosis cardíaca por transtiretina
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Pablo Garcia-Paviaa,b,c,
Corresponding author
pablogpavia@yahoo.es

Corresponding author.
, Fernando Domíngueza,b, Esther Gonzalez-Lopeza,b
a Unidad de Insuficiencia Cardíaca y Cardiopatías Familiares, Servicio de Cardiología, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
b Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain
c Universidad Francisco de Vitoria (UFV), Pozuelo de Alarcón, Spain
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Figures (2)
Tables (4)
Table 1. Comparison of the characteristics of hereditary transthyretin amyloidosis (ATTRv) and non-hereditary o wild-type (ATTRwt).
Table 2. Clinical scenarios and signs of suspicion that together with left ventricular hypertrophy should lead to consideration of transthyretin amyloidosis.
Table 3. False positives and negatives in diphosphonate scintigraphy in the diagnosis of transthyretin amyloidosis.
Table 4. Supportive and specific treatment of transthyretin CA.
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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:
Amyloidosis
Cardiac amyloidosis
Transthyretin
Heart failure
Treatment
Resumen

La amiloidosis cardíaca por transtiretina (TTR) es una enfermedad infiltrativa, grave y progresiva, que se produce por el depósito de TTR en el corazón. Puede deberse a una alteración genética en su forma hereditaria (ATTRv) o a consecuencia de un proceso degenerativo asociado a la edad (ATTRwt). Gracias a los avances en técnicas de imagen y a la posibilidad de realizar un diagnóstico no invasivo hoy conocemos que la ATTR es una enfermedad más frecuente de lo tradicionalmente considerado y que es particularmente relevante en pacientes mayores de 65 años con insuficiencia cardíaca o con estenosis aórtica. Con la aparición de opciones de tratamiento capaces de modificar la historia natural de la ATTR se hace necesario que los clínicos estén familiarizados con el proceso diagnóstico y el tratamiento de esta enfermedad. En esta revisión se repasará el espectro clínico de presentación de la ATTR, su diagnóstico y su tratamiento.

Palabras clave:
Amiloidosis
Amiloidosis cardíaca
Transtiretina
Insuficiencia cardíaca
Tratamiento

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