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About ATTR-CMAbout

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

About ATTR-CMWhat is ATTR-CM?The different types of ATTR-CM




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Diagnosis of ATTR-CMSigns and symptomsClinical cluesFormal diagnosis
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What is Transthyretin Amyloid Cardiomyopathy (ATTR-CM)?

Transthyretin Amyloid Cardiomyopathy (ATTR-CM) is a rare, and ultimately fatal disease, characterised by the deposition of amyloid fibrils in the myocardium, leading to heart failure.1,2 

Building the knowledge: Biological foundations of ATTR amyloidosis

This is a series of animated, educational modules that outline key background information on ATTR amyloidosis. This video reviews the biological foundation and disease mechanism of ATTR amyloidosis.

Symptoms onset typically over the age of 50-60 years and include:3,4

  • ​​​​​​​​​​​​​​​​​​​​​shortness of breath​​​​​​​
  • cardiac arrhythmias, particularly atrial fibrillation​​​​​​
  • heart failure
  • oedema​​​​​​
  • fatigue
  • orthostasis
  • angina

Cardiac signs include arrhythmias, thickening of the ventricular walls and interventricular septum, as well as cardiomyopathy, resulting in heart failure.4

The disease results in considerable physical and emotional impact for patients and their carers across multiple domains.​5

In the UK there is a delay to diagnosis (>4 years in 42% of cases of wild-type ATTR-CM). Barriers to diagnosis are thought to be a major reason for shortened survival with patients using hospital services a median (interquartile range) of 17 (9–27) times during the 3 years before diagnosis; by which time physical function and quality of life is greatly depleted.2

At this point, the prognosis of patients with ATTR-CM is poor; median survival in the UK varies between 2 and 5.5 years.2,6,7

The prognosis worsens with continued amyloid deposits, therefore early diagnosis is key to improving patient outcomes.1,6

Explore more Different Types of ATTR-CM Learn moreLoading See other educational resources available

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References:Witteles RM, Bokhari S, Damy T et al. Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice. JACC Heart Fail. 2019; 7(8): 709–716Lane T, Fontana M, Martinez-Naharro A et al. Natural History, Quality of Life, and Outcome in Cardiac Transthyretin Amyloidosis. Circulation. 2019; 140: 16–26.Ruberg FL and Berk JL. Transthyretin (TTR) Cardiac Amyloidosis. Circulation. 2012; 126: 1286–1300.Nativi-Nicolau J, Maurer MS. Amyloidosis cardiomyopathy: update in the diagnosis and treatment of the most common types. Curr Opin Cardiol. 2018; 33(5): 571–579.Stewart M, Shaffer S, Murphy B et al. Characterizing the High Disease Burden of Transthyretin Amyloidosis for Patients and Caregivers. Neurol Ther. 2018; 7: 349–364.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(14): 1357–1377.Grogan M, Scott CG, Kyle RA, et al. Natural History of Wild-Type Transthyretin Cardiac Amyloidosis and Risk Stratification Using a Novel Staging System. J Am Coll Cardiol. 2016; 68(10): 1014–1020.
PP-VYN-GBR-0952. February 2022
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