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Cardiovascular & Hematological Disorders-Drug Targets

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ISSN (Print): 1871-529X
ISSN (Online): 2212-4063

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Review Article

Fundamentals of Light Chain Cardiac Amyloidosis: A Focused Review

Author(s): Azka Latif*, Noman Lateef, Faryal Razzaq, Vikas Kapoor, Muhammad J. Ahsan, Muhammad Ashfaq, Ahmad Iftikhar, Faiz Anwer, Mark Holmberg and Preethi William

Volume 20, Issue 4, 2020

Page: [274 - 283] Pages: 10

DOI: 10.2174/1871529X20666201130110036

Price: $65

Abstract

The estimated prevalence of AL CA in the US is approximately 8-12 cases per million. Almost 30-50% diagnosed cases of AL amyloid in the US have multisystem involvement, including cardiac involvement. Even with the availability of advanced diagnostic testing and novel therapies, prognosis remains poor. It is overlooked as a cause of heart failure with preserved ejection fraction leading to a delay in diagnosis when management options are limited and associated with poor survival outcomes. Therefore, the education of physicians is needed to ensure that it would be highly considered as a differential diagnosis. The purpose of this manuscript is to review the advances in the diagnosis and management of cardiac amyloidosis with the aim of educating colleagues who provide care in the primary care setting. We have summarized the pathogenesis of amyloidosis, its association with plasma cell dyscrasias, novel diagnostic and surveillance approaches including echocardiography, cardiovascular magnetic resonance imaging, histopathologic techniques, systemic biomarkers, and advanced treatment approaches including supportive symptomatic management and standard of care chemotherapy targeting the amyloid deposits. Given the overall poor prognosis of amyloidosis, we have also discussed the role of palliative and hospice care.

Keywords: Cardiac amyloidosis, light chain amyloidosis, heart failure, transthyretin amyloidosis, amyloid precursors, chemotherapy, management and diagnosis of amyloidosis.

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