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Predicting which patients may have ATTR-CM can be complicated and this study attempts to develop a simple and useful way to determine which patients would benefit from further ATTR-CM testing.

Transthyretin amyloid cardiomyopathy (ATTR-CM) can drastically decrease patients’ life expectancies and quality of life, so determining which patients are at risk of this disease is a high priority, although challenging. This study, published in JAMA Cardiology, analyzed which patients with heart failure and preserved ejection fraction have sufficiently increased risk of ATTR-CM, to warrant technetium Tc 99m pyrophosphate scintigraphy (PYP). The study relied on a risk score comprised of 3 clinical factors (age, sex, and hypertension diagnosis) and 3 echocardiographic factors (ejection fraction, posterior wall thickness, and relative wall thickness). 

The retrospective cohort study comprised 666 patients with heart failure and suspected ATTR-CM referred for PYP at Mayo Clinic in Rochester, MN, from 2013 to 2020. A logistic regression model was used to predict ATTR-CM and was converted to a point-based ATTR-CM risk score. The score was subsequently analyzed and validated in a community of ATTR-CM epidemiology study of older patients with increased left ventricular wall thickness, as well as other studies. Participant cohorts included referral validation, community validation, and external validation. Precision-recall curves and predictive value versus prevalence plots indicated clinically useful classification performance for a score of 6 or more in clinically relevant ATTR-CM prevalence scenarios.

The authors conclude that the 6-variable clinical score that they developed is useful in determining which patients should receive PYP, and that it can increase recognition of ATTR-CM in patients with heart failure and preserved ejection fraction. They note further validation in larger and more diverse populations is required. 

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Davies, D. R., Redfield, M. M., Scott, C. G., Minamisawa, M., Grogan, M., Dispenzieri, A., Chareonthaitawee, P., Shah, A. M., Shah, S. J., Wehbe, R. M., Solomon, S. D., Reddy, Y. N. V., Borlaug, B. A., & AbouEzzeddine, O. F. (2022). A Simple Score to Identify Increased Risk of Transthyretin Amyloid Cardiomyopathy in Heart Failure With Preserved Ejection Fraction. JAMA Cardiol, 7(10), 1036-1044. https://doi.org/10.1001/jamacardio.2022.1781