Researchers identified factors that can accurately predict patients with wild-type transthyretin amyloid cardiomyopathy (ATTRwt) who are at high risk of early death, according to a study recently published in the Journal of the American Heart Association.
The study identified 18 variables associated with early death based on the Eastern Cooperative Oncology Group–Performance Status (ECOG‐PS) of status, age and cardiac biomarkers.
What is ATTR-CM?
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is a rare progressive disease of the heart muscle that leads to congestive heart failure. It occurs when the transthyretin protein produced by the liver is unstable. Symptoms include fatigue; shortness of breath; irregular heart rate or palpitations; swelling of the legs, ankles and stomach; brain fog; wheezing; and dizziness. It often goes underdiagnosed because of a lack of awareness and knowledge of the disease. There is currently no cure for ATTR-CM.
“In the present study, we were able to identify variables that combined in a simple staging system [that] can identify patients with cardiac ATTRwt who are at high risk of early death,” study authors said.
Read more about ATTR-CM testing and diagnosis
The authors used data from more than 600 patients with ATTR-CM who were referred to the Pavia Amyloidosis Research and Treatment Center in Pavia, Italy, between 2006 and 2021. After a 43-month follow-up, about 53% of patients had died.
An assessment of the correlation between several clinical variables and risk of early death — defined as death during the first 18 months of follow-up — identified the 18 variables associated with early death.
They included the presence of anemia, high furosemide requirements, low systolic blood pressure and low glomerular filtration fraction, N-terminal pro-brain natriuretic peptide (NT-proBNP), cardiac troponin I, age and ECOG-PS status. Subsequent statistical analysis revealed that only NT-proBNP, age, cardiac troponin I and ECOG-PS were independent predictive factors of early death.
Based on the findings, researchers divided patients into high-risk and standard-risk groups. The results showed that patients in the high-risk group had an average 17-month survival compared to patients in the standard-risk group, who had an average 57 months survival. The initiation of disease-modifying therapy with tafamidis did not appear to affect the results.
Researchers stratified patients in a risk score ranging from 0 to 5 based on NT-proBNP and cardiac troponin I levels, ECOG-PS and age. As expected, there was a significant worsening of survival with increasing stages.
None of the variables individually were enough to accurately identify an increased risk of death, study authors said. For example, although age is part of the criteria, the majority of patients over 80 survived more than 18 months.
“Conversely, the high‐risk score proposed in this study identifies subjects who have only a 45% chance of surviving 18 months and therefore are less likely to take advantage of slow‐acting therapies. Interestingly, only a minority (8%) of patients with ATTRwt are high‐risk according to the scoring system,” the authors said.
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