The use of SPECT/CT quantification of 99mTc-PYP uptake to assess tafamidis treatment response in ATTR cardiac amyloidosis

Abstract

Abstract Funding Acknowledgements Type of funding sources: None. Background/Introduction Nuclear scintigraphy with bone-avid radiotracers such as Tc-99m-PYP, Tc99m-DPC and Tc-99m-HMDP are used to diagnose transthyretin (ATTR) cardiac amyloidosis with limited quantification employing a semi-quantitative visual score and a heart to contralateral lung ratio. Therapy for ATTR cardiac amyloid currently is limited to a single FDA approved agent, tafamidis, however, there is no data regarding the longitudinal assessment of imaging findings in patients undergoing therapy. SPECT/CT acquisition and analysis with dedicated software can provide volumetric assessment and quantification of cardiac tracer uptake. Purpose The purpose of this study was to perform longitudinal assessment of cardiac ATTR amyloid 99mTc-PYP imaging findings to determine if treatment with tafamidis results in any change in quantitative measures of tracer uptake. There is no data on if treatment results in a decrease in cardiac amyloid burden as assessed by 99mTc-PYP imaging. Methods A prospective, single center, study of patients with ATTR cardiac amyloid being treated with tafamidis (either 80mg or 61mg dose) who have baseline and follow-up 99mTc-PYP studies. SPECT/CT quantification software was used to quantify heart, lung, and bone tracer uptake and generate standardized uptake values (SUVs). Comparison of baseline (before treatment) total SUVs, mean SUV value, percentage of the injected dose, mean SUV of heart to mean SUV of bone ratio and to mean SUV of right lung ratio was made to the values obtained at follow-up after tafamidis treatment. Measurements were obtained from the whole heart and the isolated left ventricle. Data was obtained by two physicians and the results averaged. Results 5 patients were analyzed with a mean age 73.8 ± 7.7, 80% were males, and a mean length of tafamidis therapy at repeat imaging of 12.5 ± 3.8 months. At follow-up there was an average decrease in whole heart total SUV counts by -25.9 ± 10.3%, in the mean SUV value by -18.2 ± 18.2% and in the percentage of injected dose by -23.7 ± 14.0%. Similar decreases were seen in the isolated left ventricle measures. Heart to bone and heart to lung ratios showed a mixed response to therapy. Detailed results are provided in the table. Conclusion The measurements of total SUV counts, mean SUV value, and percentage of the injected dose showed improvement with tafamidis treatment. The ratios did not uniformly show improvement and the reason for this finding is unclear. This new technique offers a potential method for following tafamidis therapy and assessing the cardiac amyloid burden.