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Disease Course (Progression)
The natural individual course of ATTR is difficult to assess due to the multitude of factors influencing the disease [1]; but in both ATTRwt and ATTRv, the degree of cardiac involvement is closely associated with prognosis and survival [1]
Earlier reports suggested that the median survival of patients with ATTRwt was greater than 5 years, but contemporary studies demonstrate worse outcomes with a median survival (without treatment] of 3.5 years from initial evaluation [3]; ATTRwt-CM typically progresses more slowly than ATTRv [2]
An accurate prognostic prediction method has not yet been established [4]
The following prognostic factors have been identified: age, NYHA functional class, biomarkers (e.g., BNP/NT-proBNP, troponin, renal function, uric acid), echocardiographic indices (e.g., LVEF, relative wall thickness, strain), and CMR (e.g., LGE, ECV) [4]
There are few prognostic data for Japanese patients with ATTR, and further large-scale investigations are needed [4]
Vyndaqel(tafamidis), the only disease-modifying ATTRwt therapy currently approved in Japan, demonstrated a 30% reduction in risk of death [4]
Patients’ options are limited as far as what they can do to affect their disease course
As patients experience changes that affect their functioning and independence, this can cause feelings of frustration, fear, and loss
Patients, especially those with cardiac manifestations, LIVE EACH DAY IN FEAR as they are unsure whether their organ will sustain until treatment response is achieved (if at all); all the while they live in a severely diminished physical state
Day-to-day and year-to-year uncertainty. Patients and their caregivers worry about what is to come. They don’t know the rate at which their disease will progress, or how it will progress, and their doctors cannot predict this for them either.
Taking things as they come and trying to stay positive about the future. Patients try to make the most of what they have in the “here and now”
Yilmaz, A., Bauersachs, J., Bengel, F. et al. Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK). Clin Res Cardiol 110, 479–506 (2021). https://doi.org/10.1007/s00392-020-01799-3.
Inomata T, et al. Diagnosis of wild-type transthyretin amyloid cardiomyopathy in Japan: red-!ag symptom clusters and diagnostic algorithm. ESC Heart Failure. 2021. DOI: 10.1002/ehf2.13473.
Hendren, NS, et al. Disease-Specific Biomarkers in Transthyretin Cardiac Amyloidosis. Current Heart Failure Reports. 2020;17:77-83. https://doi.org/10.1007/s11897-020-00457-z.
