Recognizing
ATTR‑CM

Transthyretin amyloid cardiomyopathy (ATTR‑CM)* is a progressive, unrelenting disease that can diminish quality of life, cause recurrent hospitalization, and lead to premature death.1–3

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

There are two subtypes of ATTR‑CM: wild-type ATTR‑CM (wtATTR‑CM) and hereditary ATTR‑CM (hATTR‑CM).*,4 These subtypes are designated by the mechanism of TTR tetramer destabilization, which occurs either through age-related changes to the protein or due to variants in the TTR gene.4

*Also known as variant ATTR‑CM (vATTR‑CM).

wild-type attr-cm versus hereditary attr-cm infographic

In a study of 879 people with ATTR-CM.11

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A prevalent pathogenic TTR gene variant is V122I, which affects 3–4% of all Black Americans and has been found in 10% of those aged 65 years or older with severe congestive heart failure14

Although predominantly diagnosed ​in men, ATTR‑CM is becoming increasingly recognized in women15

sex differences in attr-cm infographic

*In a study of 1732 consecutive patients, comprising 1095 with wild-type ATTR‑CM and 637 with hereditary ATTR‑CM.15

On average, women are 3.3 years older than men at diagnosis.15

Both sexes have similar mortality rates, 1-year disease progression rates, and overall structure and function when accounting for body size.15

References:
1.

Lane T, et al. Natural History, Quality of Life, and Outcome in Cardiac Transthyretin Amyloidosis. Circulation. 2019;140(1):16-26. 2. Rintell D, 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. 3. Nativi-Nicolau J, et al. Natural History and Progression of Transthyretin Amyloid Cardiomyopathy: Insights from ATTR-ACT. ESC Heart Fail. 2021;8(5):3875-3884. 4. Rozenbaum MH, 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. 5. Gillmore JD, et al. Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis. Circulation. 2016;133(24):2404-12. 6. Kittleson MM, et al. American Heart Association Heart Failure and Transplantation Committee of the Council on Clinical Cardiology. Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association. Circulation. 2020;142(1):e7-e22. 7. Griffin JM, et al. ATTR Amyloidosis: Current and Emerging Management Strategies: JACC: CardioOncology State-of-the-Art Review. JACC CardioOncol. 2021;3(4):488-505. 8. Witteles RM, et al. Screening for Transthyretin Amyloid Cardiomyopathy in Everyday Practice. JACC Heart Fail. 2019;7(8):709-716. 9. Gertz M, et al. Avoiding Misdiagnosis: Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Amyloidosis for the General Practitioner. BMC Fam Pract. 2020;21(1):198. 10. Reynolds MM, et al. Ocular Manifestations of Familial Transthyretin Amyloidosis. Am J Ophthalmol. 2017;183:156-162. 11. Law S, et al. Characteristics and Natural History of Early-Stage Cardiac Transthyretin Amyloidosis. Eur Heart J. 2022;43(27):2622-2632. 12. Ruberg FL, et al. Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2019;73(22):2872-2891. 13. Michels da Silva D, et al. Inflammatory and Molecular Pathways in Heart Failure-Ischemia, HFpEF and Transthyretin Cardiac Amyloidosis. Int J Mol Sci. 2019;20(9):2322. 14. Buxbaum JN, Ruberg FL. Transthyretin V122I (pV142I)* Cardiac Amyloidosis: An Age-Dependent Autosomal Dominant Cardiomyopathy Too Common to Be Overlooked as a Cause of Significant Heart Disease in Elderly African Americans. Genet Med. 2017;19(7):733-742. 15. Patel RK, et al. Sex Differences Among Patients With Transthyretin Amyloid Cardiomyopathy – From Diagnosis to Prognosis. Eur J Heart Fail. 2022;24(12):2355-2363.