1. Fundamentals of Light Chain Cardiac Amyloidosis: A Focused Review
- Author
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Muhammad Junaid Ahsan, Mark Holmberg, Noman Lateef, Muhammad Ashfaq, Ahmad Iftikhar, Azka Latif, Preethi William, Faryal Razzaq, Vikas Kapoor, and Faiz Anwer
- Subjects
medicine.medical_specialty ,Heart Diseases ,Amyloid ,medicine.medical_treatment ,Paraproteinemias ,Dyscrasia ,medicine ,Animals ,Humans ,Immunoglobulin Light-chain Amyloidosis ,Intensive care medicine ,Pharmacology ,Chemotherapy ,business.industry ,Myocardium ,Amyloidosis ,Disease Management ,Hematology ,General Medicine ,medicine.disease ,Magnetic Resonance Imaging ,Cardiac amyloidosis ,Echocardiography ,Heart failure ,Molecular Medicine ,Differential diagnosis ,Cardiology and Cardiovascular Medicine ,business ,Heart failure with preserved ejection fraction ,Biomarkers - 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.
- Published
- 2021
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