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About ATTR-CMAbout

Learn about ATTR-CM and uncover the differences between hereditary and wild-type ATTR-CM

Types of ATTR-CM

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Click here for Vyndaqel®▼ (tafamidis) Prescribing Information.

What is Transthyretin Amyloid Cardiomyopathy (ATTR-CM)? Impact on the heart1 Normal, healthy heart

ATTR-CM heart with thickened walls caused by increased amyloid fibril deposition

Illustrative representation.

 Mode of Disease

ATTR amyloidosis is due to the misfolding of transthyretin (TTR) protein2

TTR is a transport protein for a thyroid hormone (thyroxine [T4]) and is a retinol-binding protein.3

  • Produced mainly in the liver, but there is also some production in the choroid plexus (brain) and retinal epithelium (eye)3
  • TTR protein plays an important role in the body. The long-term impact of TTR reduction in humans is unknown3,4
  • The complete absence of TTR in humans has not been reported3


 

The physiologic role of TTR is not fully defined, but the growing body of evidence suggests TTR has additional important functions.3

 

 The pathogenesis of ATTR amyloidosis is caused by the destabilisation, not production, of TTR protein tetramers.3

Adapted from Castano et al. American College of Cardiology 2015.5

 Main types of cardiac amyloidosis

98% of currently diagnosed cardiac amyloidosis cases result from fibrils composed of ATTR or monoclonal immunoglobulin light chains (immunoglobulin light chain amyloidosis, or AL).7

ATTR-CM is one of the most common types of cardiac amyloidosis8-10

  • wtATTR-CM is idiopathic and is not considered to be a hereditary disease11,12
  • hATTR-CM occurs due to a mutation in the transthyretin (TTR) gene11
It is important to rule out AL amyloidosis by testing for the presence of monoclonal protein via serum and urine immunofixation and serum-free light chain assay.
  • VYNDAQEL® 61 mg (tafamidis) is indicated for the treatment of wild‑type or hereditary transthyretin amyloidosis in adult patients with cardiomyopathy (ATTR-CM).
  • Amyloid light chain (AL) amyloidosis must be excluded before starting treament with VYNDAQEL.

It is important to differentiate between ATTR and AL as they have distinct clinical courses and require different approaches to management  Explore Types of ATTR-CM Loading Fibrils can deposit in multiple areas.6 

Because amyloid fibrils can deposit in different parts of the body, including the heart and nerves, some patients may present with a mixed phenotype, namely, symptoms of both cardiomyopathy and polyneuropathy.3,6 Suspicion of cardiac involvement should prompt immediate cardiac evaluation to assess for ATTR-CM.7 

This mode of disease underscores the important function of TTR protein and the consequence of destabilisation for your patients.

 A multidisciplinary approach

A well-coordinated multidisciplinary health care team can increase early and accurate diagnosis of ATTR-CM.13

ATTR-CM is characterised by multiorgan involvement and can manifest in many ways.14,6  

It is important to include a multidisciplinary health care team in the diagnostic process. Coordinated care can help ensure that the patient receives the right diagnosis.7

AL= immunoglobulin light chain amyloidosis; (wt/h)ATTR-CM= (wild type/hereditary) transthyretin amyloid cardiomyopathy; TTR=transthyretin.

References:Pinney JH, Whelan CJ, Petrie A, et al. Senile systemic amyloidosis: clinical features at presentation and outcome. J Am Heart Assoc. 2013;2:e000098;Coelho T, Ericzon B, Falk R, et al. A Guide to Transthyretin Amyloidosis. Clarkston, MI: Amyloidosis Foundation; 2018. https://amyloidosis.org/sites/default/files/pdf-docs/pages/resources/2023-03/2018%20ATTR.pdf [Last accessed January 2024];Liz MA, Coelho T, Bellotti V, Fernandez-Arias MI, Mallaina P, Obici L. A narrative review of the role of transthyretin in health and disease. Neurol Ther. 2020;9:395–402;Adams D, Ando Y, Beirão JM, et al. Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy. J Neurol. 2021;268: 2109 – 2122;Castano A, Narotsky D, Maurer MS. Emerging Therapies for Transthyretin Cardiac Amyloidosis Could Herald a New Era for the Treatment of HFPEF. Expert Analysis. American College of Cardiology. October 14, 2015. Available at: https://www.acc.org/Latest-in-Cardiology/Articles/2015/10/13/08/35/Emerging-Therapies-for-Transthyretin-Cardiac-Amyloidosis [Last accessed January2024];Maurer MS, Bokhari S, Damy T, et al. Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis. Circ Heart Fail. 2019;12:e006075;Garcia–Pavia P, Rapezzi C, Adler Y, et al. Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2021;42:1554–1568; Maurer MS, Elliott P, Comenzo R, Semigran M, Rapezzi C. Addressing common questions encountered in the diagnosis and management of cardiac amyloidosis. Circulation. 2017;135:1357–1377; 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;Narotsky DL, Castaño A, Weinsaft JW, Bokhari S, Maurer MS. Wild-type transthyretin cardiac amyloidosis: novel insights from advanced imaging. Can J Cardiol. 2016;32:1166.e1-1166.e10;Benson MD, Buxbaum JN, Eisenberg DS, et al. Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee. Amyloid. 2018;25:215–219;Connors LH, Sam F, Skinner M, et al. Heart failure resulting from age-related cardiac amyloid disease associated with wild-type transthyretin: a prospective, observational cohort study. Circulation. 2016;133:282–290;Rapezzi C, Merlini G, Quarta CC, et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009;120:1203–1212;Witteles RM, Bokhari S, Damy T et al. Screening for transthyretin amyloid cardiomyopathy in everyday practice. JACC Heart Fail. 2019;7(8):709-716.doi:10.1016/j.jchf.2019.04.010.Explore more Explore available supporting resources Supporting Resources Loading Discover more about ATTR-CM Types of ATTR-CM Loading What imaging modalities help raise suspicion of ATTR-CM? Suspect ATTR-CM Loading
PP-VYN-GBR-1134. February 2024

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