The Present and Future
State-of-the-Art Review
AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

https://doi.org/10.1016/j.jacc.2016.06.053Get rights and content
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Abstract

The amyloidoses are a group of protein-folding disorders in which ≥1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease. The last decade has seen considerable progress in understanding the amyloidoses. In this review, current and novel approaches to the diagnosis and treatment of cardiac amyloidosis are discussed, with particular reference to AL amyloidosis in the heart.

Key Words

cardiomyopathy
multiple myeloma
TTR amyloidosis

Abbreviations and Acronyms

AL amyloidosis
light-chain amyloidosis
ATTR amyloidosis
transthyretin amyloidosis
ATTRm amyloidosis
mutant transthyretin amyloidosis
ATTRwt
wild-type transthyretin amyloidosis
CMR
cardiac magnetic resonance
CPHPC
(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid
DPD
2,3-dicarboxypropane-1, 1-diphosphonate
ECG
electrocardiography
LGE
late gadolinium enhancement
LV
left ventricular
MGUS
monoclonal protein of unknown significance
NT-proBNP
N-terminal pro–B-type natriuretic peptide
SAP
serum amyloid P component
TcPYP
technetium pyrophosphate
TTR
transthyretin

Cited by (0)

This study was supported in part by the Demarest Lloyd Jr. Foundation, The Friends of Burt Glazov Cardiac Amyloidosis Fund, and the Harold Grinspoon Charitable Foundation.

Dr. Falk receives funds for consulting from Ionis Pharmaceuticals and Alnylam Pharmaceuticals; and research support from GlaxoSmithKline. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Listen to this manuscript's audio summary by JACC Editor-in-Chief Dr. Valentin Fuster.

© 2016 by the American College of Cardiology Foundation. Published by Elsevier.