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J Am Coll Cardiol. 2019 Jun 11;73(22):2872-2891. doi: 10.1016/j.jacc.2019.04.003.

Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review.

Author information

1
Section of Cardiovascular Medicine, Department of Medicine, Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts.
2
Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
3
Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio.
4
Departments of Chemistry and Molecular Medicine, Scripps Research Institute, La Jolla, California.
5
Division of Cardiology, Department of Medicine, Center for Advanced Cardiac Care, Columbia University Medical Center, New York, New York. Electronic address: msm10@cumc.columbia.edu.

Abstract

Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) in older adults, resulting from myocardial deposition of misfolded transthyretin (TTR) or pre-albumin. Characteristic patterns of echocardiography and cardiac magnetic resonance can strongly suggest the disease but are not diagnostic. The diagnosis can be made with noninvasive nuclear imaging when there is no evidence of a monoclonal protein. Amyloid fibril formation results from a destabilizing mutation in hereditary ATTR amyloidosis (hATTR) or from an aging-linked process in wild-type ATTR amyloidosis (wtATTR). Recent studies have suggested that up to 10% to 15% of older adults with HF may have unrecognized wtATTR. Associated features, including carpal tunnel syndrome and lumbar spinal stenosis, raise suspicion and may afford a means for early diagnosis. Previously treatable only by organ transplantation, pharmaceutical therapy that slows or halts ATTR-CM progression and favorably affects clinical outcomes is now available. Early recognition remains essential to afford the best treatment efficacy.

KEYWORDS:

amyloidosis; cardiomyopathy; transthyretin

PMID:
31171094
DOI:
10.1016/j.jacc.2019.04.003

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