Lily K Stern1, Michelle M Kittleson2,3. 1. Department of Cardiology, Smidt Heart Institute, Cedars-Sinai, Los Angeles, CA, USA. 2. Department of Cardiology, Smidt Heart Institute, Cedars-Sinai, Los Angeles, CA, USA. Michelle.kittleson@cshs.org. 3. , Los Angeles, USA. Michelle.kittleson@cshs.org.
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.
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.
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