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AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review

Amyloid light chain (AL) amyloidosis is a rare, debilitating, often fatal disease. Symptoms of cardiomyopathy are common presenting features, and patients often are referred to cardiologists. Cardiac amyloid infiltration is the leading predictor of death. However, the variable presentation and perce...

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Detalles Bibliográficos
Autores principales: Wechalekar, Ashutosh D., Fontana, Marianna, Quarta, C. Cristina, Liedtke, Michaela
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9700258/
https://www.ncbi.nlm.nih.gov/pubmed/36444232
http://dx.doi.org/10.1016/j.jaccao.2022.08.009
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author Wechalekar, Ashutosh D.
Fontana, Marianna
Quarta, C. Cristina
Liedtke, Michaela
author_facet Wechalekar, Ashutosh D.
Fontana, Marianna
Quarta, C. Cristina
Liedtke, Michaela
author_sort Wechalekar, Ashutosh D.
collection PubMed
description Amyloid light chain (AL) amyloidosis is a rare, debilitating, often fatal disease. Symptoms of cardiomyopathy are common presenting features, and patients often are referred to cardiologists. Cardiac amyloid infiltration is the leading predictor of death. However, the variable presentation and perceived rarity of the disease frequently lead to delay in suspecting amyloidosis as a cause of heart failure, leading to misdiagnoses and a marked delay in diagnosis, with devastating consequences for the patient. A median time from symptom onset to correct diagnosis of about 2 years is often too long when median survival from diagnosis for patients with AL amyloidosis and cardiomyopathy is 4 months to 2 years. The authors highlight the challenges to diagnosis, identify gaps in the current knowledge, and summarize novel treatments on the horizon to raise awareness about the critical need for early recognition of symptoms and diagnosis of AL amyloidosis aimed at accelerating treatment and improving outcomes for patients.
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spelling pubmed-97002582022-11-27 AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review Wechalekar, Ashutosh D. Fontana, Marianna Quarta, C. Cristina Liedtke, Michaela JACC CardioOncol Mini-Focus Issue: Amyloidosis Amyloid light chain (AL) amyloidosis is a rare, debilitating, often fatal disease. Symptoms of cardiomyopathy are common presenting features, and patients often are referred to cardiologists. Cardiac amyloid infiltration is the leading predictor of death. However, the variable presentation and perceived rarity of the disease frequently lead to delay in suspecting amyloidosis as a cause of heart failure, leading to misdiagnoses and a marked delay in diagnosis, with devastating consequences for the patient. A median time from symptom onset to correct diagnosis of about 2 years is often too long when median survival from diagnosis for patients with AL amyloidosis and cardiomyopathy is 4 months to 2 years. The authors highlight the challenges to diagnosis, identify gaps in the current knowledge, and summarize novel treatments on the horizon to raise awareness about the critical need for early recognition of symptoms and diagnosis of AL amyloidosis aimed at accelerating treatment and improving outcomes for patients. Elsevier 2022-11-15 /pmc/articles/PMC9700258/ /pubmed/36444232 http://dx.doi.org/10.1016/j.jaccao.2022.08.009 Text en © 2022 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Mini-Focus Issue: Amyloidosis
Wechalekar, Ashutosh D.
Fontana, Marianna
Quarta, C. Cristina
Liedtke, Michaela
AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review
title AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review
title_full AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review
title_fullStr AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review
title_full_unstemmed AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review
title_short AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review
title_sort al amyloidosis for cardiologists: awareness, diagnosis, and future prospects: jacc: cardiooncology state-of-the-art review
topic Mini-Focus Issue: Amyloidosis
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9700258/
https://www.ncbi.nlm.nih.gov/pubmed/36444232
http://dx.doi.org/10.1016/j.jaccao.2022.08.009
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