Noninvasive Identification of ATTRwt Cardiac Amyloid: The Re-emergence of Nuclear Cardiology

Mathew S Maurer, Mathew S Maurer

Abstract

More than half of all subjects with chronic heart failure are older adults with preserved ejection fraction (HFpEF). Effective therapy for this condition is yet to be delineated by clinical trials, suggesting that a greater understanding of underlying biologic mechanisms is needed, especially for the purpose of clinical intervention and future clinical trials. Amyloid infiltration of the myocardium is an underappreciated contributing factor to HFpEF that is often caused by misfolded monomers or oligomers of the protein transthyretin. While previously called senile cardiac amyloidosis and traditionally requiring endomyocardial biopsy for diagnosis, advances in our pathophysiologic understanding of this condition, coupled with nuclear imaging techniques using bone isotopes that can diagnose this condition noninvasively and the development of potential therapies, have resulted in a renewed interest in this previously considered "rare" condition. This reviewer focuses on the re-emergence of nuclear cardiology using pyrophosphate agents that hold promise for early, noninvasive identification of affected individuals.

Keywords: Aging; Cardiac amyloid; Senile cardiac amyloid; Technetium pyrophosphate.

Conflict of interest statement

Conflict of Interest: Dr. Maurer has been a paid consultant to Pfizer, Inc, Alnylam Pharmaceuticals, Inc. Prothena, Inc and ISIS Pharmaceuticals. His institution (Columbia University Medical Center) has received funding for the conduct of clinical trials from Pfizer, Inc. and Alnylam Pharmaceuticals.

Dr. Maurer drafted the manuscript and had control over all aspects of the review.

Copyright © 2015 Elsevier Inc. All rights reserved.

Figures

Figure
Figure
Tc-PYP99 myocardial planar scanning in a patient with biopsy proven AL amyloid (upper panel) with sternal but no myocardial uptake and in a patient with biopsy proven TTR cardiac amyloid (lower panel) in which there is diffuse myocardial uptake.

Source: PubMed

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