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Review
. 2021 Mar 16;23(4):47.
doi: 10.1007/s11912-021-01028-8.

Updates in Cardiac Amyloidosis Diagnosis and Treatment

Lily K Stern  1 , Michelle M Kittleson  2   3
Affiliations
Review

Updates in Cardiac Amyloidosis Diagnosis and Treatment

Lily K Stern et al. Curr Oncol Rep. .

Abstract

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 99mtechnetium (99mTc) 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.

Trial registration: ClinicalTrials.gov NCT03860935.

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

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References

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