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Amiloidosis cardíaca. De la fisiopatología al tratamiento inicial

Jorge Thierer, Federico Bonora

Revista del Consejo Argentino de Residentes de Cardiología ;():0018-0025 


El término amiloidosis fue acuñado por Rudolf Virchow en 1854 cuando al examinar muestras de un material hialino al microscopio notó su semejanza con el almidón (amilus en latín), y por ello lo llamó amiloide (semejante al almidón).
Esta entidad es una de las enfermedades sistémicas llamadas enfermedades por depósito. En particular, se caracteriza por depósito extracelular de una sustancia llamada amiloide y toma relevancia para el cardiólogo por su afectación cardíaca, que ocasiona efectos deletéreos en este órgano tales como insuficiencia cardíaca, arritmias o enfermedad valvular.
Las dos formas más frecuentes de amiloidosis primaria son la amiloidosis de cadenas livianas (AL) y la amiloidosis por transtirretina (ATTR), que a su vez se subdivide en la forma mutante (ATTRm) y la forma salvaje (wild type, ATTRwt).
Hasta hace poco tiempo el tratamiento cardiovascular se basaba en el tratamiento de sostén y carecíamos de drogas que modificaran sustancialmente el pronóstico; hoy en día, sin duda, el advenimiento de nuevas opciones terapéuticas empieza a demostrar la capacidad de mejorar el pronóstico de una enfermedad cuyo diagnóstico implicaba elevada mortalidad a corto plazo.
Este primer artículo revisará conceptos básicos, ahondará en la fisiopatología de las diferentes variantes de la patología y hará consideraciones iniciales sobre el diagnóstico y el pronóstico.


Palabras clave: amiloidosis, afectación cardiovascular, fisiopatología, síntomas, diagnóstico,

The term amyloidosis was coined by Rudolf Virchow in 1854, because when examining samples of hyaline material under a microscope, he noticed its similarity to starch (Latin amilus), and therefore called it amyloid (similar to starch).
This entity is one of the systematic diseases called deposit diseases, particularly characterized by extracellular deposit of a substance called amyloid,which becomes relevant for the cardiologist for its cardiac involvement causing deleterious effects on this organ, such as heart failure, arrhythmias or valve disease.
The two most common forms of primary amyloidosis are light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), which in turn is subdivided into the mutant form (ATTRm) and the wild type (ATTRwt).
Until recently, cardiovascular treatment was based on supportive care and we lacked drugs that substantially modified prognosis. Nowadays, without a doubt, the advent of new therapeutic options is beginning to demonstrate the ability to improve the prognosis of a disease, whose diagnosis implied high short-term mortality.
This first article will review basic concepts, delve into the pathophysiology of the different pathology variants, and present initial considerations on diagnosis and prognosis.


Keywords: amyloidosis, cardiovascular involvement, pathophysiology, symptoms, diagnosis,


Los autores declaran no poseer conflictos de intereses.

Fuente de información Consejo Argentino de Residentes de Cardiología. Para solicitudes de reimpresión a Revista del CONAREC hacer click aquí.

Recibido 2020-02-20 | Aceptado 2020-03-01 | Publicado 2020-04-30

Tabla 1. Formas más frecuentes de amiloidosis cardíaca primaria.

Figura 1. La combinación de estos hallazgos, principalmente en hombres >60 años o mujeres >70 año...

Tabla 2. Marcadores pronósticos en amiloidosis.

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Autores

Jorge Thierer
Unidad Insuficiencia Cardíaca.
Federico Bonora
Médico Residente de Cardiología. CEMIC, CABA, Rep. Argentina.

Autor correspondencia

Federico Bonora
Médico Residente de Cardiología. CEMIC, CABA, Rep. Argentina.

Correo electrónico: fede_bonora@hotmail.com

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Titulo
Amiloidosis cardíaca. De la fisiopatología al tratamiento inicial

Autores
Jorge Thierer, Federico Bonora

Publicación
Revista del CONAREC

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Consejo Argentino de Residentes de Cardiología

Fecha de publicación
2020-04-30

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