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Updates in Cardiac Amyloidosis Diagnosis and Treatment

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CURRENT ONCOLOGY REPORTS
卷 23, 期 4, 页码 -

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SPRINGER
DOI: 10.1007/s11912-021-01028-8

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Cardiac amyloidosis; Transthyretin amyloidosis; Light chain amyloidosis; Technetium pyrophosphate scan; Monoclonal light chains; Tafamidis

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Cardiac amyloidosis is an underrecognized cause of heart failure. Recent advances in diagnosis and treatment include accurate noninvasive diagnostic modalities and effective therapies for both transthyretin and light chain amyloidosis. Early recognition and implementation of these advancements are crucial in minimizing morbidity and mortality.
Purpose of Review Cardiac amyloidosis is an underrecognized cause of heart failure. We review clinical clues to the diagnoses, a rational approach to testing, and current and emerging therapies. Recent Findings Advances in the diagnosis of amyloid cardiomyopathy include (1) use of (99m)technetium (Tc-99m) bone-avid compounds which allow accurate noninvasive diagnosis of transthyretin cardiac amyloidosis (ATTR-CM) in the context of a negative monoclonal light chain screen; and (2) the use of serum and urine immunofixation electrophoresis with serum free light chains as an accurate first diagnostic step for light chain cardiac amyloidosis (AL-CM). Advances in treatment include tafamidis for ATTR-CM and immunologic therapies for AL-CM. With the advent of accurate noninvasive diagnostic modalities and effective therapies, early recognition of cardiac amyloidosis is paramount to implement a diagnostic algorithm and expeditiously institute effective therapies to minimize morbidity and mortality.

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