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Rev Esp Cardiol (Engl Ed). 2017 Nov;70(11):991-1004. doi: 10.1016/j.rec.2017.05.036. Epub 2017 Sep 1.

Diagnosis and Treatment of Transthyretin Cardiac Amyloidosis. Progress and Hope.

[Article in English, Spanish]

Author information

1
Unidad de Insuficiencia Cardiaca y Cardiopatías Familiares, Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain.
2
Unidad de Insuficiencia Cardiaca y Cardiopatías Familiares, Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain.
3
Unidad de Insuficiencia Cardiaca y Cardiopatías Familiares, Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain; Facultad de Medicina, Universidad Francisco de Vitoria, Madrid, Spain. Electronic address: pablogpavia@yahoo.es.

Abstract

Cardiac amyloidosis is an infiltrative disorder caused by extracellular protein deposition. Transthyretin is a proamyloidotic protein that produces one of the most frequent forms of cardiac amyloidosis, either through mutations or a wild-type form (previously known as senile amyloidosis). Until very recently, diagnosis of transthyretin amyloidosis (ATTR) was very uncommon and histological confirmation was mandatory, making diagnosis of ATTR a real challenge in daily clinical practice. Moreover, the specific therapeutic options to alter the clinical course of the disease were very limited. However, advances in cardiac imaging and diagnostic strategies have improved recognition of ATTR. In addition, several compounds able to modify the natural history of the disease are in the final phases of research, with promising results. Given that effective therapies are on the horizon, cardiologists should be well-versed in this disease and be familiar with its diagnosis and treatment. This review describes the broad clinical spectrum of ATTR in detail, as well as recent advances in the diagnosis and treatment of this condition.

KEYWORDS:

Amiloidosis cardiaca; Cardiac amyloidosis; Hereditaria; Hereditary; Senil; Senile; Transthyretin; Transtiretina

PMID:
28870641
DOI:
10.1016/j.rec.2017.05.036
[Indexed for MEDLINE]

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