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

Learn about the red flags that may raise suspicion of and techniques used to diagnose ATTR-CM

Explore a range of educational resources to support the diagnosis of ATTR-CM

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The different types of Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

Transthyretin Amyloid Cardiomyopathy (ATTR-CM) falls under the disease group cardiac amyloidosis. The most common types of cardiac amyloidosis, accounting for 95% of cases, are ATTR and Immunoglobulin Light Chain Amyloidosis (AL). It is important to differentiate between ATTR and AL, as they have distinct clinical courses and require different approaches to management.1

There are two types of ATTR-CM – Wild-type ATTR-CM and Hereditary ATTR-CM2,3
​​​​​​​

Building the knowledge: Navigating the ATTR amyloidosis disease landscape

This is a series of animated, educational modules that outline key background information on ATTR amyloidosis. This video reviews the disease landscape, and highlights differences between wtATTR amyloidosis and hATTR amyloidosis.

Wild-type ATTR-CM vs. hereditary ATTR-CM

Wild-type ATTR-CM

Hereditary ATTR-CM

Wild-type ATTR-CM is idiopathic and is not considered to be a hereditary disease.4 It is thought to account for the majority of all ATTR-CM cases.5

Hereditary ATTR-CM in the UK there are thought to be around 40-50 new cases each year5

Wild-type ATTR-CM is also known as:4

Hereditary ATTR-CM is also known as:3,10
  • Senile cardiac amyloidosis
  • Senile systemic amyloidosis
  • Age-related amyloidosis
  • Variant ATTR (ATTRv)
  • Mutant ATTR (ATTRm)
  • Familial amyloidosis
| Patient Considerations​​​​​​​
| ​​​​​​​Patient Considerations
  • Ethnicity: predominantly white4
  • Found mostly in men4
  • Symptoms onset typically over the age of 60 years6

  • Wild-type ATTR-CM frequently presents with:
    •  ​​​​​​​Heart failure with preserved ejection fraction4
    • Cardiac arrhythmias, particularly atrial fibrillation7​​​​​​​
    • A history of bilateral carpal tunnel syndrome8
  • Men and women11
  • Age of symptom onset depends on the mutation but may occur as early as 50-60 years6
  • ​​​​​​​Wild-type ATTR-CM frequently presents with:
    • ​​​​​​​Heart failure with preserved ejection fraction11-13
    • A range of neurological symptoms (peripheral and autonomic)11-13
    • Gastrointestinal symptoms11-13​​​​​​​
| Prognosis
| Prognosis
Median survival: ~3.5 years9
Median survival: 2-5.5 years 5,14

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 Learn about the signs and symptoms of ATTR-CM

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References:
  1. Donnelly JP, Hanna M. Cardiac amyloidosis: An update on diagnosis and treatment. Cleve Clin J Med. 2017; 84(12 Suppl 3): 12–2.
  2. Rapezzi C, Merlini G, Quarta CC, et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009; 120(13): 1203–1212.
  3. Benson MD, Buxbaum JN, Eisenberg DS, et al. Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee. Amyloid. 2018; 25(4): 215–219
  4. 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.
  5. 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.
  6. Ruberg FL and Berk JL. Transthyretin (TTR) Cardiac Amyloidosis. Circulation. 2012; 126: 1286–1300. 
  7. 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.
  8. Sperry BW, Reyes BA, Ikram A, et al. Tenosynovial and Cardiac Amyloidosis in Patients Undergoing Carpal Tunnel Release. J Am Coll Cardiol. 2018; 72(17): 2040–2050.
  9. 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.
  10. Tuzovic M, Yang EH, Baas AS, et al. Cardiac Amyloidosis: Diagnosis and Treatment Strategies. Curr Oncol Rep. 2017; 19(7): 46.
  11. Maurer MS, Hanna M, Grogan M, et al. Genotype and Phenotype of Transthyretin Cardiac Amyloidosis. J Am Coll Cardiol. 2016; 68(2): 161–172.
  12. Coelho T, Maurer MS, Suhr OB. THAOS – The Transthyretin Amyloidosis Outcomes Survey: initial report on clinical manifestations in patients with hereditary and wild-type transthyretin amyloidosis. Curr Med Res Opin 2013;29:63–76.
  13. González-López E, López-Sainz Á, Garcia-Pavia P. Diagnosis and Treatment of Transthyretin Cardiac Amyloidosis. Progress and Hope. Rev Esp Cardiol (Engl Ed). 2017;70(11):991‐1004.
  14. 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.
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