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Diagnosing cardiac amyloidosis in every-day practice: A practical guide for the cardiologist

Maria Papathanasiou1,2, Alexander Carpinteiro3,2, Christoph Rischpler4,2

  • 1Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Hufelandstrasse 55, 45147 Essen, Germany.

International Journal of Cardiology. Heart & Vasculature
|May 7, 2020
PubMed

Insights

Cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure. This review focuses on diagnosing CA, particularly transthyretin (ATTR-CA), using current and emerging methods for better patient outcomes.

Area of Science:

  • Cardiology
  • Nephrology
  • Hematology

Background:

  • Cardiac amyloidosis (CA) is an increasingly recognized cause of heart failure and mortality.
  • Underdiagnosis stems from low awareness and non-specific symptoms.
  • CA involves extracellular deposition of misfolded protein fibrils, primarily transthyretin (ATTR) or immunoglobulin light chains (AL).

Purpose of the Study:

  • To provide an overview of cardiac amyloidosis.
  • To highlight current and emerging diagnostic modalities for CA.
  • To present a diagnostic algorithm for suspected CA in clinical practice.

Main Methods:

  • Literature review of CA prevalence, symptoms, and causes.
  • Analysis of diagnostic tools, including imaging and biopsy.
  • Development of a practical diagnostic algorithm.

Main Results:

  • ATTR-CA is prevalent in older heart failure patients, while AL-CA is rarer.
  • Early and accurate diagnosis is crucial for effective management.
  • Emerging diagnostic techniques show promise for improved detection.

Conclusions:

  • CA is a significant contributor to heart failure that requires increased clinical awareness.
  • A systematic diagnostic approach is essential for timely identification.
  • The proposed algorithm aids in evaluating patients with suspected cardiac amyloidosis.

Keywords:
99mTc-DPD, 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acidAA, amyloid A amyloidosisAApoA-1, apolipoprotein A-1 amyloidosisAL, light chain amyloidosisATTR, transthyretin amyloidosisATTRv, variant transthyretin amyloidosisATTRwt, wild type transthyretin amyloidosisAmyloidosisCA, cardiac amyloidosisCardiomyopathyECV, Extracellular volumeEMB, endomyocardial biopsyHeart failureLGE, late gadolinium enhancementLV, left ventricular/ left ventricularLight chainsMGUS, monoclonal gammopathy of undetermined significanceMRI, magnetic resonance imagingNT-proBNP, N-terminal pro B-type natriuretic peptidePET, positron-emission tomographySPECT, single photon emission computed tomographyTransthyretin

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