Updates in Cardiac Amyloidosis Diagnosis and Treatment

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.

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.