Heart with magnifying glass revealing a maze in a ventricle, alongside text reading 'ATTR-CM suspect & detect, uncover the clues for diagnosis'

Home > What is Transthyretin Amyloid Cardiomyopathy (ATTR-CM)?

 

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

        
         Watch this video to understand the mechanism of disease of ATTR-CM

The disease results in considerable physical and emotional impact for patients and their carers across multiple domains.2

Symptoms usually start over the age of 60 and include:3,4

  • shortness of breath
  • cardiac arrhythmias, particularly atrial fibrillation
  • oedema
  • fatigue
  • fainting
  • angina

Cardiac signs include arrhythmias, progressive thickening of the ventricular walls and interventricular septum, as well as cardiomyopathy, resulting in heart failure.5

In the UK there is a delay to diagnosis (>4 years in nearly half of cases). 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.6

At this point, the prognosis of patients with ATTR-CM is poor; median survival in the UK varies between 2 and 3.5 years.7,8,9,10

Therefore, understanding the disease, early identification, diagnosis, and treatment are critical.4


| Investigating infiltrative cardiac disease as a subset of hypertrophic cardiomyopathy

Dr Gerald Carr-White of Guy’s and St Thomas’ Hospital, London summarises his symposium speech, where he discusses the diagnosis of hypertrophic cardiomyopathy and identifies useful diagnostic clues.

Dr Gerald Carr-White, Consultant Cardiologist, Guy’s and St Thomas’ Hospital, London

This symposium at the BCS Annual Conference 2019 was an educational symposium organised and sponsored by Pfizer.
The content of the symposium was developed and agreed by the faculty.

 

NEXT SECTION: The different types of ATTR-CM >

 


 

Contact a member of the Pfizer team here

References

1. Maurer MS, Hanna M, Grogan M et al. Genotype and Phenotype of Transthyretin Cardiac Amyloidosis. J Am Coll Cardiol. 2016; 68(2): 161–172. 
2. 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.
3. Ruberg FL and Berk JL. Transthyretin (TTR) Cardiac Amyloidosis. Circulation. 2012; 126: 1286–1300. 
4. Witteles RM, Bokhari S, Damy T et al. Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice. JACC Heart Fail. 2019; 7(8): 709–716. 
5. Lopez EG, Gagliardi C, Dominguez F et al. Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths. Eur Heart J. 2017; 38: 1895–1904. 
6. Lane T, Fontana M, Martinez-Naharro A et al. Natural History, Quality of Life, and Outcome in Cardiac Transthyretin Amyloidosis. Circulation. 2019; 140: 16–26. 
7. Connors LH, Sam F, Skinner M et al. Heart Failure Due to Age-Related Cardiac Amyloid Disease Associated With Wild-Type Transthyretin: A Prospective, Observational Cohort Study. Circulation. 2016; 133(3): 282–290. 
8. Pinney JH, Whelan CJ, Petrie A et al. Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome. J Am Heart Assoc. 2013; 2(2): e000098. 
9. Maurer MS, Elliott P, Comenzo R et al. Addressing Common Questions Encountered in the Diagnosis and Management of Cardiac Amyloidosis. Circulation. 2017; 135(14): 1357–1377.
10. 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. 

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