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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 are ATTR and Immunoglobulin Light Chain Amyloidosis (AL).1–4 It is important to differentiate between ATTR and AL, as they have distinct clinical courses4 and require different approaches to management.5

There are two types of ATTR-CM – Wild-type ATTR-CM and Hereditary ATTR-CM4,6

Diagram showing the most common types of cardiac amyloidosis: trasthyretin amyloidosis (ATTR) and immunoglobin light chain amyloidosis (AL)

| Investigating cardiac amyloidosis

Dr Carol Whelan from the National Amyloidosis Centre in London summarises her symposium speech, explaining the importance of accurate diagnosis for patients with these conditions and the implications for their subsequent management and treatment.

Dr Carol Whelan, Consultant Cardiologist, National Amyloidosis Centre, Royal Free 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.


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


Wild-type ATTR-CM


Hereditary ATTR-CM

    Wild-type ATTR-CM is idiopathic7 and is not considered to be a hereditary disease.6 It is thought to account for the majority of all ATTR-CM cases.8

    Wild-type ATTR-CM is also known as:7,9

    • Senile cardiac amyloidosis
    • Senile systemic amyloidosis
    • Age-related amyloidosis

    | Patient Considerations

    • Ethnicity: predominantly white7,9
    • Mostly men7,9,10
    • Symptom onset typically over the age of 60 years8
    • Heart failure with preserved ejection fraction7,9,10
    • Cardiac arrhythmias, particularly atrial fibrillation7,9,10
    • History of bilateral carpal tunnel syndrome11

    | Prognosis

    Median survival: ~3.5 years7,10,12

    Hereditary ATTR-CM in the UK there are thought to be around 30 new cases each year.13

    Hereditary ATTR-CM is also known as:

    • Variant ATTR (ATTRv)6
    • ATTRm6
    • Mutant ATTR3
    • Hereditary amyloidosis14
    • Familial amyloidosis15

    | Patient Considerations

    • Men and women9
    • Symptom onset may occur as early as 50-60 years of age8,16
    • Heart failure with preserved ejection fraction9
    • Neurological symptoms (peripheral and autonomic)9
    • Gastrointestinal symptoms9
    • History of bilateral carpal tunnel syndrome11

    | Prognosis

    Median survival: 2-3 years2


    NEXT SECTION: Diagnosis of ATTR-CM >




    1. 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(9): 1166.e1–1166.e10. 
    2. 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. 
    3. 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. 
    4. 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. 
    5. Donnelly JP, Hanna M. Cardiac amyloidosis: An update on diagnosis and treatment. Cleve Clin J Med. 2017; 84(12 Suppl 3): 12–26. 
    6. 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. 
    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. Ruberg FL and Berk JL. Transthyretin (TTR) Cardiac Amyloidosis. Circulation. 2012; 126: 1286–1300. 
    9. Maurer MS, Hanna M, Grogan M, et al. Genotype and Phenotype of Transthyretin Cardiac Amyloidosis. J Am Coll Cardiol. 2016; 68(2): 161–172. 
    10. 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. 
    11. 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. 
    12. 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. 
    13. 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. 
    14. Tuzovic M, Yang EH, Baas AS, et al. Cardiac Amyloidosis: Diagnosis and Treatment Strategies. Curr Oncol Rep. 2017; 19(7): 46. 
    15. Dubrey SW, Hawkins PN, Falk RH. Amyloid diseases of the heart: assessment, diagnosis, and referral. Heart. 2011; 97(1): 75–84.
    16. Rapezzi C, Quarta CC, Riva L, et al. Transthyretin-related amyloidoses and the heart: a clinical overview. Nat Rev Cardiol. 2010; 7(7): 398–408. 

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