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Vol. 77. Issue 1.
Pages 79-87 (January 2024)
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Vol. 77. Issue 1.
Pages 79-87 (January 2024)
Review article
Monitoring cardiac amyloidosis with multimodality imaging
Monitorización de la amiloidosis cardiaca con imagen multimodal
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Neasa Starr, Adam Ioannou, Ana Martinez-Naharro
Corresponding author
anamartinez.naharro@nhs.net

Corresponding author.
National Amyloidosis Centre, Royal Free Hospital NHS Foundation Trust, London, United Kingdom
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Table 1. Summary of cardiovascular imaging modalities for determining prognosis in ATTR-CA and AL-CA
Table 2. Summary of cardiovascular imaging modalities for disease surveillance in ATTR-CA and AL-CA
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Abstract

Cardiac amyloidosis (CA) refers to an infiltrative process involving amyloid fibril deposition in the myocardium causing restrictive cardiomyopathy. While various types can affect the heart, the predominant forms are immunoglobulin light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. This review article explores the expanding field of imaging techniques used to diagnose AL-CA and ATTR-CA, highlighting their usefulness in prognostication and disease surveillance. Echocardiography is often the initial imaging modality to suspect CA and, since the incorporation of nonbiopsy criteria using bone scintigraphy, diagnosing ATTR-CA has become more attainable following exclusion of plasma cell dyscrasia. Cardiac magnetic resonance is progressively emerging as a vital tool for imaging CA, and is used in diagnosis, prognostication, and disease surveillance. The use of cardiac magnetic resonance in AL-CA is discussed, as it has been shown to accurately evaluate organ response to chemotherapy. As novel drug treatments emerge in the realm of ATTR-CA, the use of cardiovascular imaging surveillance to monitor disease progression is discussed, as it is gaining prominence as a critical consideration. The ongoing phase III trials investigating treatments for patients with ATTR-CA, will undoubtedly enhance our understanding of cardiac imaging surveillance.

Keywords:
Cardiac amyloidosis
Cardiac magnetic resonance
Restrictive cardiomyopathy
Abbreviations:
AL
ATTR
ATTRv
ATTRwt
CA
CMR
ECV
GLS
Resumen

La amiloidosis cardiaca (AC), tanto en amiloidosis de cadenas ligeras de inmunoglobulina (AL) como en amiloidosis por transtirretina (ATTR), es la principal causa de morbimortalidad por amiloidosis sistémica. Por ello, la imagen cardiaca es un aspecto crucial en el diagnóstico y la monitorización de estos pacientes, en especial la ecocardiografía, la resonancia magnética cardiaca y la gammagrafía ósea. La monitorización de la respuesta al tratamiento en la ATTR-CA mediante imagen cardiaca se ha empleado principalmente en ensayos clínicos aleatorizados que investigan nuevos fármacos para esta enfermedad. El conocimiento acerca del seguimiento en ATTR-AC por imagen cardiaca indudablemente mejorará con los estudios en fase III actualmente en curso. En los últimos años, la utilidad de la resonancia magnética cardiaca en la evaluación de la respuesta al tratamiento en AL-AC ha aumentado considerablemente y se han incrementado la comprensión de la respuesta individual al tratamiento, el conocimiento del proceso subyacente a la enfermedad y, por lo tanto, la mejora de las estrategias terapéuticas. El objetivo de esta revisión es explorar el papel de la imagen cardiaca en el diagnóstico y sobre todo la monitorización de la AC.

Palabras clave:
Amiloidosis cardiaca
Resonancia magnética cardiaca
Miocardiopatía restrictiva

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