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Amyloidosis in Cardiology Practice: A Race Against Time

  • Authors: Mathew Maurer, MD; Jose Nativi-Nicolau, MD; Ronald M. Witteles, MD
  • CME / ABIM MOC / CE Released: 3/28/2019
  • Valid for credit through: 3/28/2020, 11:59 PM EST
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Target Audience and Goal Statement

This activity is intended for cardiologists, neurologists, hematology and oncology specialists, and nurses.

The goal of this activity is to improve the recognition, diagnosis, and management of cardiac amyloidosis.

Upon completion of this activity, participants will:

  • Have increased knowledge regarding the
    • Clinical signs and symptoms of amyloidosis
    • Latest clinical data on treatment of hereditary transthyretin-related amyloidosis
  • Have greater competence related to
    • Patient assessment to improve early diagnosis of amyloidosis


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  • Mathew Maurer, MD

    Arnold and Arlene Goldstein Professor of Cardiology
    Department of Medicine Cardiology
    Columbia University Medical Center
    New York, New York


    Disclosure: Mathew Maurer, MD, has disclosed the following relevant financial relationships:
    Served as an advisor or consultant for: Akcea Therapeutics; Amylon Therapeutics; Ionis Pharmaceutcials; Pfizer Inc.


  • Jose Nativi-Nicolau, MD

    Assistant Professor
    University of Utah
    Salt Lake City, Utah


    Disclosure: Jose Nativi-Nicolau, MD, has disclosed the following relevant financial relationships:
    Served as an advisor or consultant for: Akcea Therapeutics; Alnylam Pharmaceuticals, Inc.; Pfizer Inc.
    Received grants for clinical research from: Akcea Therapeutics; Eidos Therapeutics; Pfizer Inc.

  • Ronald M. Witteles, MD

    Associate Professor of Cardiovascular Medicine
    Stanford Amyloid Center
    Stanford University School of Medicine
    Stanford, California


    Disclosure: Ronald M. Witteles, MD, has disclosed the following relevant financial relationships:
    Served as an advisor or consultant for: Alnylam Pharmaceuticals, Inc.; Pfizer Inc.
    Received grants for clinical research from: Eidos Therapeutics; Pfizer Inc.


  • Joy P. Marko, MS, APN-C, CCMEP

    Scientific Director, Medscape, LLC


    Disclosure: Joy P. Marko, MS, APN-C, CCMEP, has disclosed no relevant financial relationships.

  • Kalanethee Paul-Pletzer, PhD

    Scientific Director, Medscape, LLC


    Disclosure: Kalanethee Paul-Pletzer, PhD, has disclosed no relevant financial relationships.

Nurse Planner

  • Amy Bernard, MS, BSN, RN-BC, CHCP

    Lead Nurse Planner, Medscape, LLC


    Disclosure: Amy Bernard, MS, BSN, RN-BC, CHCP, has disclosed no relevant financial relationships.

Peer Reviewer

This activity has been peer reviewed and the reviewer has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Abbott Laboratories; Medtronic, Inc.

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Amyloidosis in Cardiology Practice: A Race Against Time


Patient Assessment and Diagnosis of Amyloidosis


  • Patient Assessment and Diagnosis of Amyloidosis

  • Slide 1.

    Slide 1.

    (Enlarge Slide)
  • Cardiac Manifestations [1]

    • Systolic dysfunction manifests later in the disease course
    • Heart block is extremely common, particularly in ATTR
    • Many patients, particularly with ATTR, do not have low voltages on ECG
    • Rather than low QRS voltages alone, the abnormal ratio between LV thickness and QRS voltages is characteristic of CA
    • An LVH pattern seen on echocardiography that is not hypertrophy of the muscle but rather amyloid deposition
    • An elevated troponin level is a common finding due to chronic gradual destruction of cardiomyocytes
    • New-onset atrial fibrillation with no AV nodal blocking agents and antiarrhythmic agents and with a heart rate of 60 to 70 beats/min is another characteristic feature of CA

  • Slide 2.

    Slide 2.

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  • Biopsy: Gold Standard and Only Way to Diagnose AL Amyloidosis [1,16]

    • Abnormal light chains or an abnormal serum protein immunoelectrophoresis (SPIE) is not sufficient to make the diagnosis of AL amyloidosis
    • Abdominal fat pad biopsy is most frequently performed but has mediocre sensitivity
    • A negative fat pad biopsy result does not rule out AL amyloidosis

  • Slide 3.

    Slide 3.

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  • Adjunctive Laboratory Tests [1]

    • Free light-chain assay and SPIE are the most important tests
    • Free light-chain assay
      • A κ:λ ratio of greater than 2:1 (in either direction) strongly implies clonal production of κ or λ light chains
    • SPIE
      • Directly tests for clonality
      • Most patients with plasma cell dyscrasias secrete a whole immunoglobulin and a light chain

  • Slide 4.

    Slide 4.

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  • 99mTechnetium-PYP Imaging [2]

    • 99mTc-pyrophosphate (PYP) cardiac imaging distinguishes AL amyloidosis from cardiac ATTR
    • May be a simple, widely available method for identifying subjects with cardiac ATTR

  • Slide 5.

    Slide 5.

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  • 99mTc-PYP Imaging: Assessments [17]

    • Cardiac retention is assessed with a
      • Semiquantitative visual score and
      • Quantitative heart:contralateral (H:CL) ratio
    • Timing of imaging is important
      • H:CL ratio is higher with 1-hour vs 3-hour incubation

  • Slide 6.

    Slide 6.

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  • Diagnostic Value of Bone Scintigraphy [18]

    • Bone scintigraphy enables the diagnosis of cardiac ATTR to be made reliably without the need for histology in patients who do not have a monoclonal gammopathy
    • Bone scintigraphy should not be used before ruling out monoclonal protein

  • Slide 7.

    Slide 7.

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  • Diagnostic Algorithm

    • In patients with clinical suspicion of amyloidosis, the first step is to rule out/rule in monoclonal gammopathy
    • If the monoclonal protein test is positive, the next step is biopsy
    • If the monoclonal protein test is negative, the next step is bone scintigraphy
    • If bone scintigraphy is positive, the next step is genetic testing to identity the type of ATTR

  • Slide 8.

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