Managing ATTR-CM

Early diagnosis and intervention are critical to slowing ATTR-CM progression and prolonging quality of life.1-3

Diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM)* has exponentially increased over the last decade due to enhanced awareness, changes in the standard of care, and the development of a noninvasive diagnostic algorithm.1,4,5 These developments have significantly improved survival, but many people continue to manage cardiac morbidity and diminishing quality of life.4,6,7 More effective treatment options are needed.

*Also known as ATTR cardiomyopathy, or ATTR cardiac amyloidosis (ATTR-CA).

Infographic summarizing symptom duration, disease severity, and survival around ATTR‑CM diagnosis

In 2012, 99mTc-DPD scintigraphy became a routine part of the diagnostic algorithm for ATTR‑CM.4

†Median survival during 2002 to 2012 ranged from 35 to 39 months and during 2012 to 2021 from 53 to >60 months.8

Treating ATTR-CM

Learn about a TTR stabilizer prescription treatment option.9

As understanding of ATTR‑CM progression and opportunities for earlier intervention have grown, goals for patient management have expanded beyond prolonging survival to include1,10:

  • Maintaining quality of life10
  • Preserving independence with valve replacement10
  • Keeping patients out of the hospital10
  • Stabilization or improvement of prognostic biomarkers10
Summary of contemporary measures used to assess ATTR‑CM

Access patient profiles and compare individual characteristics.

Patient Profiler Tool

The progression of ATTR-CM deprives people of their strength, mobility, and independence. At later stages of disease, those affected may lose their ability to engage in previously enjoyable activities or perform routine activities such as household chores and self-care.4,12-14

Common clinical measures of functional capacity and quality of life for people with ATTR-CM include: ​

6-Minute Walk Distance (6MWD)10,15,16

Functional capacity correlates with quality of life and prognosis

  • A practical measure of functional capacity that correlates with quality of life and prognosis
  • Declines in 6MWD are associated with increased levels of NT-proBNP, a prognostic biomarker for ATTR-CM progression

The Kansas City Cardiomyopathy
Questionnaire (KCCQ)4

A 23-item questionnaire that examines:​

  • Physical function
  • Independence
  • Symptom frequency and severity
  • Social function
  • Limitations to quality of life

6MWD=6-minute walk distance; KCCQ-OS=Kansas City Cardiomyopathy Questionnaire Overall Summary; NT-proBNP=N-terminal pro B-type natriuretic peptide; TTR=transthyretin.

Keep in touch

Sign up to receive the latest
information about ATTR-CM.

Stay informed
References:
1.

Kittleson MM, Maurer MS, Ambardekar AV, et al. Cardiac amyloidosis: evolving diagnosis and management: a scientific statement from the American Heart Association. Circulation. 2020;142(1):e7-e22. 2. Rozenbaum MH, Large S, Bhambri R, et al. Impact of delayed diagnosis and misdiagnosis for patients with transthyretin amyloid cardiomyopathy (ATTR-CM): a targeted literature review. Cardiol Ther. 2021;10(1):141-159. 3. Rozenbaum MH, Large S, Bhambri R, et al. Estimating the health benefits of timely diagnosis and treatment of transthyretin amyloid cardiomyopathy. J Comp Eff Res. 2021;10(11):927-938. 4. Lane T, Fontana M, Martinez-Naharro A, et al. Natural history, quality of life, and outcome in cardiac transthyretin amyloidosis. Circulation. 2019;140(1):16-26. 5. Gillmore JD, Maurer MS, Falk RH, et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation. 2016;133(24):2404-2412. 6. Miller M, Pal A, Albusairi W, et al. Enthalpy-driven stabilization of transthyretin by AG10 mimics a naturally occurring genetic variant that protects from transthyretin amyloidosis. J Med Chem. 2018;61(17):7862-7876. 7. Hanna M, Damy T, Grogan M, et al. Impact of tafamidis on health-related quality of life in patients with transthyretin amyloid cardiomyopathy (from the tafamidis in transthyretin cardiomyopathy clinical trial). Am J Cardiol. 2021;141:98-105. 8. Ioannou A, Patel RK, Razvi Y, et al. Impact of earlier diagnosis in cardiac ATTR amyloidosis over the course of 20 years. Circulation. 2022;146(22):1657-1670. 9. Morfino P, Aimo A, Vergaro G, et al. Transthyretin stabilizers and seeding inhibitors as therapies for amyloid transthyretin cardiomyopathy.  Pharmaceutics. 2023;15(4):1129. 10. Garcia-Pavia P, Rapezzi C, Adler Y, et al. Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur J Heart Fail. 2021;23(4):512-526. 11. Hanson JLS, Arvanitis M, Koch CM, et al. Use of serum transthyretin as a prognostic indicator and predictor of outcome in cardiac amyloid disease associated with wild-type transthyretin. Circ Heart Fail. 2018;11(2):e004000. 12. Rintell D, Heath D, Braga Mendendez F, et al. Patient and family experience with transthyretin amyloid cardiomyopathy (ATTR-CM) and polyneuropathy (ATTR-PN) amyloidosis: results of two focus groups. Orphanet J Rare Dis. 2021;16(1):70. 13. Stewart M, Shaffer S, Murphy B, et al. Characterizing the high disease burden of transthyretin amyloidosis for patients and caregivers. Neurol Ther. 2018;7(2):349-364. 14. Nativi-Nicolau J, Judge DP, Hoffman JE, et al. Natural history and progression of transthyretin amyloid cardiomyopathy: insights from ATTR-ACT. ESC Heart Fail. 2021;8(5):3875-3884. 15. Solway S, Brooks D, Lacasse Y, Thomas S. A qualitative systematic overview of the measurement properties of functional walk tests used in the cardiorespiratory domain. Chest. 2001;119(1):256-270. 16. ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002;166(1):111-117.