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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


Amyloidosis in Cardiology Practice: A Race Against Time


  • Amyloidosis in Cardiology Practice: A Race Against Time

  • Slide 1.

    Slide 1.

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  • Program Overview

    • CA is an underappreciated and underdiagnosed cause of heart failure (HF)
    • It is attributable to extracellular deposition of fibrillary proteins

  • Slide 3.

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  • Epidemiology and Clinical Presentation of Amyloidosis With a Focus on Cardiac Manifestations

  • Slide 4.

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  • CA: Epidemiology [1]

    • There are 2 pathophysiologic substrates for CA [2]
      • Immunoglobulin light-chain (AL) amyloidosis (also known as primary amyloidosis): the amyloid fibrils are composed of immunoglobulin light chains, produced by a clonal population of plasma cells in the bone marrow
      • Transthyretin-related amyloidosis (ATTR): misfolded monomers or dimers of the normally tetrameric transthyretin (TTR) protein, a transport protein synthesized in the liver
      • 2 types of ATTR:
        • Hereditary ATTR (hATTR; also known as familial ATTR or mutant ATTR [ATTRmt])
        • Wild-type ATTR (ATTRwt, also known as senile systemic amyloidosis)
    • ATTRmt is caused by more than 100 mutations in the TTR protein that are inherited in an autosomal dominant fashion and can affect individuals of all ages
    • ATTRwt is predominantly described in older men

  • Slide 5.

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  • Identifying Populations at Risk: Older Adults -- THAOS Registry [3]

    • THOAS is a global registry of ATTR (ATTRmt and ATTRwt)
    • ATTRwt most common form in US adults

  • Slide 6.

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  • Prevalence of ATTRwt: Almost Doubles Every 5 Years From Age 75 [4]

    • Myocardial uptake of bone tracers (bone scintigraphy) has emerged as useful tool for the early detection of ATTR
    • Myocardial uptake in bone scan is very prevalent with aging

  • Slide 7.

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  • Identifying Populations at Risk: Patients With HFpEF [5]

    • ATTRwt is an underdiagnosed cause of HFpEF
    • ATTRwt can be diagnosed noninvasively with scintigraphy

  • Slide 8.

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  • Identifying Populations at Risk: Patients With Severe AS Undergoing TAVR [6]

    • Cardiac ATTR is prevalent in 16% of patients with severe calcific aortic stenosis (AS) undergoing transcatheter aortic valve replacement (TAVR)
    • It is associated with a severe AS phenotype of low-flow low-gradient with mildly reduced ejection fraction (EF)

  • Slide 9.

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  • Identifying Populations at Risk: Afro-Caribbean Patients With HF [7]

    • In Afro-Caribbean patients, ATTR V122I is an underappreciated cause of HF
    • Cardiomyopathy is often misattributed to hypertension

  • Slide 10.

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  • Most Common Symptoms of CA [8]

    • Patients with CA present with multiple nonspecific symptoms
    • A high suspicion index is required to facilitate early diagnosis
    • Carpel tunnel (CT) syndrome (especially bilateral), spinal stenosis, and atrial fibrillation (especially ablation refractory) are 3 red flags for amyloidosis in cardiac patients

  • Slide 11.

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  • Diagnostic Tools: ECG [9]

    • Low voltage is classically reported as an electrocardiographic (ECG) finding in CA
    • Low voltage is a relatively late finding in CA and may not be useful for early identification

  • Slide 12.

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  • Diagnostic Tools: Cardiac MRI [10]

    • Global transmural or subendocardial late gadolinium enhancement (LGE) is most common, but focal patchy LGE is also observed in CA
    • Focal patchy LGE may not be labeled as CA

  • Slide 13.

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  • Diagnostic Tools: Echocardiography [11]

    • Echocardiography is the cornerstone for the diagnosis and management of patients with CA
    • Increased left ventricular (LV) wall thickness, often with a restrictive filling pattern, is a classic feature of advanced CA
    • Misdiagnosis as true LV hypertrophy (LVH) is frequent

  • Slide 14.

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  • CA: Survival [12,13]

    • HF in ATTR progresses slowly, whereas HF in AL amyloidosis progresses rapidly
    • AL amyloidosis is a medical emergency
    • Echocardiographic results cannot distinguish between AL amyloidosis and ATTR

  • Slide 15.

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  • Patient Journey: Survey of Patients With AL Amyloidosis [1]

    • CA is often misdiagnosed
    • Delayed diagnosis has significant consequences for patients

  • Slide 16.

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