Maurer MS, Elliott P, Comenzo R, et al. Addressing common questions encountered in the diagnosis and management of cardiac amyloidosis. Circulation. 2017;135:1357-1377.
Bokhari S, Castaño A, Pozniakoff T, et al. (99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses. Circ Cardiovasc Imaging. 2013;6:195-201.
Maurer MS, Hanna M, Grogan M, et al. Genotype and Phenotype of Transthyretin Cardiac Amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey). J Am Coll Cardiol. 2016;68:161-172.
Mohamed-Salem L, Santos-Mateo JJ, Sanchez-Serna J, et al. Prevalence of wild type ATTR assessed as myocardial uptake in bone scan in the elderly population. Int J Cardiol. 2018;270:192-196.
González-López E, Gallego-Delgado M, Guzzo-Merello G, et al. Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J. 2015;36:2585-2594.
Castaño A, Narotsky DL, Hamid N, et al. Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement. Eur Heart J. 2017;38:2879-2887.
Dungu JN, Papadopoulou SA, Wykes K, et al. Afro-Caribbean heart failure in the United Kingdom: cause, outcomes, and ATTR V122I cardiac amyloidosis. Circ Heart Fail. 2016;9.pii:e003352.
Nativi-Nicolau J, Maurer MS. Amyloidosis cardiomyopathy: update in the diagnosis and treatment of the most common types. Curr Opin Cardiol. 2018;33:571-579.
Cyrille NB, Goldsmith J, Alvarez J, et al. Prevalence and prognostic significance of low QRS voltage among the three main types of cardiac amyloidosis. Am J Cardiol. 2014;114:1089-1093.
Syed IS, Glockner JF, Feng D, et al. Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis. JACC Cardiovasc Imaging. 2010;3:155-164.
Di Nunzio D, Recupero A, de Gregorio C, et al. Echocardiographic findings in cardiac amyloidosis: inside two-dimensional, Doppler, and strain imaging. Curr Cardiol Rep. 2019;21:7.
Ng B, Connors LH, Davidoff R, et al. Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis. Arch Intern Med. 2005;165:1425-1429.
Dubrey SW, Cha K, Skinner M, et al. Familial and primary (AL) cardiac amyloidosis: echocardiographically similar diseases with distinctly different clinical outcomes. Heart. 1997;78:74-82.
Kumar S, Dispenzieri A, Lacy MQ, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol. 2012;30:989-995.
Brunjes DL, Castano A, Clemons A, et al. Transthyretin cardiac amyloidosis in older Americans. J Card Fail. 2016;22:996-1003.
Bogov B, Lubomirova M, Kiperova B. Biopsy of subcutaneous fatty tissue for diagnosis of systemic amyloidosis. Hippokratia. 2008;12:236-239.
Castano A, Haq M, Narotsky DL, et al. Multicenter study of planar technetium 99m pyrophosphate cardiac imaging: predicting survival for patients with ATTR cardiac amyloidosis. JAMA Cardiol. 2016;1:880-889.
Gillmore JD, Maurer MS, Falk RH, et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation. 2016;133:2404-2412.
Penchala SC, Connelly S, Wang Y, et al. AG10 inhibits amyloidogenesis and cellular toxicity of the familial amyloid cardiomyopathy-associated V122I transthyretin. Proc Natl Acad Sci U S A. 2013;110:9992-9997.
Adams D, Gonzalez-Duarte A, O'Riordan WD, et al. Patisiran, an RNAi therapeutic, for hereditary transthyretin amyloidosis. N Engl J Med. 2018;379:11-21.
Benson MD, Waddington-Cruz M, Berk JL, et al. Inotersen treatment for patients with hereditary transthyretin amyloidosis. N Engl J Med. 2018;379:22-31.
Solomon SD, Adams D, Kristen A, et al. Effects of patisiran, an RNA interference therapeutic, on cardiac parameters in patients with hereditary transthyretin-mediated amyloidosis. Circulation. 2019;139:431-443.
Maurer MS, Schwartz JH, Gundapaneni B, et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy. N Engl J Med. 2018;379:1007-1016.

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Moderator

Mathew Maurer, MD

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

Disclosures

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.

Panelists

Jose Nativi-Nicolau, MD

Assistant Professor
University of Utah
Salt Lake City, Utah

Disclosures

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
Co-Director
Stanford Amyloid Center
Stanford University School of Medicine
Stanford, California

Disclosures

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.

Editors

Joy P. Marko, MS, APN-C, CCMEP

Scientific Director, Medscape, LLC

Disclosures

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

Kalanethee Paul-Pletzer, PhD

Scientific Director, Medscape, LLC

Disclosures

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

Disclosures

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.

CME / ABIM MOC / CE

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
THIS ACTIVITY HAS EXPIRED FOR CREDIT
Valid for credit through: 3/28/2020

Start Activity

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

Disclosures

Moderator

Mathew Maurer, MD

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

Disclosures

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.

Panelists

Jose Nativi-Nicolau, MD

Assistant Professor
University of Utah
Salt Lake City, Utah

Disclosures

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
Co-Director
Stanford Amyloid Center
Stanford University School of Medicine
Stanford, California

Disclosures

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.

Editors

Joy P. Marko, MS, APN-C, CCMEP

Scientific Director, Medscape, LLC

Disclosures

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

Kalanethee Paul-Pletzer, PhD

Scientific Director, Medscape, LLC

Disclosures

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

Disclosures

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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Latest Advances in the Treatment of hATTR

« Back

Latest Advances in the Treatment of hATTR
[ CLOSE WINDOW ]

Slide 1.

Strategies to Prevent TTR Amyloid Deposition ^[19]
- Stabilize the TTR tetramer
- Reduce the production of TTR by controlling gene expression
- In clinical trials, reducing protein expression by 80% to 85% is not associated with clinical consequences
[ CLOSE WINDOW ]

Slide 2.

APOLLO: Study Design ^[20]
- APOLLO is a multicenter, international, randomized, double-blind, placebo-controlled, phase 3 trial
- Key inclusion criteria:
  - A Neuropathy Impairment Score (NIS) of 5 to 130 (with higher scores indicating more impairment)
  - A polyneuropathy disability score of IIIb or lower (with higher scores indicating more impaired walking ability)
  - Adequate liver and renal function
- Key exclusion criteria:
  - Previous liver transplantation or planning to undergo liver transplantation
  - New York Heart Association (NYHA) class of III or IV
[ CLOSE WINDOW ]

Slide 3.

APOLLO: Primary Endpoint – mNIS+7 ^[20]
- Patisiran improved neuropathy
- The effect of patisiran was seen as early as 9 months
[ CLOSE WINDOW ]

Slide 4.

NEURO-TTR: Study Design ^[21]
- NEURO-TTR is an international, randomized, double-blind, placebo-controlled, phase 3 trial
- Key inclusion criterion:
  - An NIS of 10 to 130
- Key exclusion criteria:
  - Clinically significant abnormalities in screening laboratory values
  - Karnofsky performance status score of 50 or less (with lower scores indicating greater disability)
  - Other causes of polyneuropathy besides hATTR
  - Previous liver transplantation
  - NYHA class III or higher
[ CLOSE WINDOW ]

Slide 5.

NEURO-TTR: Primary Endpoint – mNIS+7 ^[21]
- Inotersen improved the course of neurologic disease and quality of life in patients
- Enhanced monitoring required for adverse events
[ CLOSE WINDOW ]

Slide 6.

APOLLO: Cardiac Subgroup: NT-proBNP ^[22]
- Patisiran improved cardiac manifestations of hATTR:
  - Improved cardiac structure and function
  - Reduced N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels
[ CLOSE WINDOW ]

Slide 7.

ATTR-ACT: Study Design ^[23]
- ATTR-ACT is a multicenter, international, double-blind, placebo-controlled, phase 3 trial
- Key inclusion criteria included confirmation of cardiac involvement:
  - Echocardiography, with an end-diastolic interventricular septal wall thickness exceeding 12 mm
  - History of HF, with at least 1 prior hospitalization or clinical evidence of HF
  - NT-proBNP level greater than or equal to 600 pg/mL
  - 6-minute walk-test (6MWT) distance exceeding 100 m
- Key exclusion criteria:
  - NYHA class IV HF
  - History of liver or heart transplantation
  - An implanted cardiac device
[ CLOSE WINDOW ]

Slide 8.

ATTR-ACT: All-Cause Mortality ^[23]
- Tafamidis was associated with a 30% lower all-cause mortality compared with placebo
[ CLOSE WINDOW ]

Slide 9.

ATTR-ACT: CV-Related Hospitalizations ^[23]
- Tafamidis was associated with a 32% lower rate of cardiovascular (CV)-related hospitalizations
[ CLOSE WINDOW ]

Slide 10.

ATTR-ACT: 6MWT Distance ^[23]
- At month 30, tafamidis was also associated with a lower rate of decline in distance for the 6MWT
[ CLOSE WINDOW ]

Slide 11.

ATTR-ACT: Primary Endpoints Stratified by Baseline NYHA Class ^[23]
- Patients with NYHA class I/II derived greater benefit from tafamidis than did patients with NYHA class III
[ CLOSE WINDOW ]

Slide 12.

Summary -- Changing Landscape of ATTR
[ CLOSE WINDOW ]

Slide 13.

Thank You
[ CLOSE WINDOW ]

Slide 14.

This content has been condensed for improved clarity.

Educational Impact Challenge

What did you learn from this activity? Please click on the "Next" button to proceed to a brief survey to see how your knowledge improved after the education. You can also see how your answers compare with those of your peers.

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Page 5 of 7

Educational Impact Challenge

CME / ABIM MOC / CE Information Download Slides

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References

Faculty and Disclosures

Moderator

Mathew Maurer, MD

Panelists

Jose Nativi-Nicolau, MD

Ronald M. Witteles, MD

Editors

Joy P. Marko, MS, APN-C, CCMEP

Kalanethee Paul-Pletzer, PhD

Nurse Planner

Amy Bernard, MS, BSN, RN-BC, CHCP

Peer Reviewer

CME / ABIM MOC / CE

Amyloidosis in Cardiology Practice: A Race Against Time

Target Audience and Goal Statement

Disclosures

Moderator

Mathew Maurer, MD

Panelists

Jose Nativi-Nicolau, MD

Ronald M. Witteles, MD

Editors

Joy P. Marko, MS, APN-C, CCMEP

Kalanethee Paul-Pletzer, PhD

Nurse Planner

Amy Bernard, MS, BSN, RN-BC, CHCP

Peer Reviewer

Accreditation Statements

For Physicians

For Nurses

Instructions for Participation and Credit

Amyloidosis in Cardiology Practice: A Race Against Time

Latest Advances in the Treatment of hATTR

Latest Advances in the Treatment of hATTR

Slide 1.

Strategies to Prevent TTR Amyloid Deposition [19]

Slide 2.

APOLLO: Study Design [20]

Slide 3.

APOLLO: Primary Endpoint – mNIS+7 [20]

Slide 4.

NEURO-TTR: Study Design [21]

Slide 5.

NEURO-TTR: Primary Endpoint – mNIS+7 [21]

Slide 6.

APOLLO: Cardiac Subgroup: NT-proBNP [22]

Slide 7.

ATTR-ACT: Study Design [23]

Slide 8.

ATTR-ACT: All-Cause Mortality [23]

Slide 9.

ATTR-ACT: CV-Related Hospitalizations [23]

Slide 10.

ATTR-ACT: 6MWT Distance [23]

Slide 11.

ATTR-ACT: Primary Endpoints Stratified by Baseline NYHA Class [23]

Slide 12.

Summary -- Changing Landscape of ATTR

Slide 13.

Thank You

Slide 14.

Educational Impact Challenge

Strategies to Prevent TTR Amyloid Deposition ^[19]

APOLLO: Study Design ^[20]

APOLLO: Primary Endpoint – mNIS+7 ^[20]

NEURO-TTR: Study Design ^[21]

NEURO-TTR: Primary Endpoint – mNIS+7 ^[21]

APOLLO: Cardiac Subgroup: NT-proBNP ^[22]

ATTR-ACT: Study Design ^[23]

ATTR-ACT: All-Cause Mortality ^[23]

ATTR-ACT: CV-Related Hospitalizations ^[23]

ATTR-ACT: 6MWT Distance ^[23]

ATTR-ACT: Primary Endpoints Stratified by Baseline NYHA Class ^[23]