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

Frederick L. Ruberg, Martha Grogan, Mazen Hanna, Jeffery W. Kelly, Mathew S. Maurer

Research output: Contribution to journalReview article

5 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)2872-2891
Number of pages20
JournalJournal of the American College of Cardiology
Volume73
Issue number22
DOIs
StatePublished - Jun 11 2019

Fingerprint

Prealbumin
Amyloidosis
Cardiomyopathies
Amyloid
Heart Failure
Familial Amyloidosis
Spinal Stenosis
Carpal Tunnel Syndrome
Organ Transplantation
Echocardiography
Early Diagnosis
Albumins
Magnetic Resonance Spectroscopy
Mutation
Pharmaceutical Preparations
Proteins
Therapeutics

Keywords

  • amyloidosis
  • cardiomyopathy
  • transthyretin

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Transthyretin Amyloid Cardiomyopathy : JACC State-of-the-Art Review. / Ruberg, Frederick L.; Grogan, Martha; Hanna, Mazen; Kelly, Jeffery W.; Maurer, Mathew S.

In: Journal of the American College of Cardiology, Vol. 73, No. 22, 11.06.2019, p. 2872-2891.

Research output: Contribution to journalReview article

Ruberg, Frederick L. ; Grogan, Martha ; Hanna, Mazen ; Kelly, Jeffery W. ; Maurer, Mathew S. / Transthyretin Amyloid Cardiomyopathy : JACC State-of-the-Art Review. In: Journal of the American College of Cardiology. 2019 ; Vol. 73, No. 22. pp. 2872-2891.
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