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Imaging Options in Cardiac Amyloidosis: Differentiating AL from ATTR

  • Sumeet Pawar
  • Muhammad Haq
  • Frederick L. Ruberg
  • Edward J. MillerEmail author
Emerging Debates and Concepts (V Dilsizian and T Schindler, section editors)
Part of the following topical collections:
  1. Topical Collection on Emerging Debates and Concepts

Abstract

Purpose of Review

Cardiac amyloidosis (CA) results from the myocardial deposition of abnormally folded endogenous proteins leading to significant morbidity and mortality. Accurate and timely non-invasive diagnosis of cardiac amyloidosis and the correct identification of amyloid type are critical for choosing appropriate therapy. Disease type is defined by the precursor protein and most typically involves light chain (AL) or transthyretin (ATTR). Traditionally, cardiac amyloidosis has been suspected on the basis of characteristic echocardiographic features and confirmed with endomyocardial biopsy. Recent advances in echocardiography, cardiovascular magnetic resonance (CMR), and nuclear imaging now permit the non-invasive diagnosis of CA with a high degree of confidence and, in some cases, assignment of amyloid types.

Recent Findings

CMR techniques including late gadolinium enhancement and new protocols such as T1 mapping and extra-cellular volume (ECV) quantification have afforded insight into the diagnosis and pathophysiology of cardiac amyloidosis, while bone-avid nuclear tracers can identify ATTR with a high degree of accuracy.

Summary

We highlight the recent advances in imaging techniques that can identify cardiac amyloidosis with a special emphasis on nuclear modalities used for differentiating light chain from transthyretin disease.

Keywords

Cardiac amyloidosis Multimodality imaging Transthyretin Light chain amyloidosis 

Notes

Compliance with Ethical Standards

Conflict of Interest

Sumeet Pawar, Muhammad Haq, Frederick L. Ruberg, and Edward J. Miller declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Sumeet Pawar
    • 1
  • Muhammad Haq
    • 2
  • Frederick L. Ruberg
    • 3
  • Edward J. Miller
    • 4
    Email author
  1. 1.Department of MedicineBoston University School of Medicine and Boston Medical CenterBostonUSA
  2. 2.Department of Internal MedicineBeth Israel Deaconess Medical Center, Harvard Medical SchoolBostonUSA
  3. 3.Department of Medicine, Section of Cardiovascular Medicine, Amyloidosis CenterBoston University School of Medicine and Boston Medical CenterBostonUSA
  4. 4.Section of Cardiovascular MedicineYale University School of MedicineNew HavenUSA

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