Background Differentiating immunoglobulin light-chain (AL) from transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, therapy, and genetic counseling. We validated the discriminatory ability of 99mTc-pyrophosphate scintigraphy (99mTc-PYP) in AL vs. TTR-related cardiac amyloidoses. Methods and Results 45 subjects (12 AL, 16 ATTR wild-type, and 17 ATTR mutants) underwent 99mTc-PYP planar and single-photon positive emission computed tomography (SPECT) cardiac imaging. Scans were performed by experienced nuclear cardiologists blinded to the subjects’ cohort assignment. Cardiac retention was assessed with both a semi-quantitative visual score (range 0, no uptake to 3, diffuse uptake) and by quantitative analysis by drawing a region of interest (ROI) over the heart corrected for contralateral counts and calculating a heart-to-contralateral ratio (H/CL). Subjects with ATTR cardiac amyloid had a significantly higher semi-quantitative cardiac visual score than the AL cohort (2.9±0.06 vs. 0.8±0.27, p<0.0001) as well as a higher quantitative score (1.80±0.04 vs.1.21±0.04, p<0.0001). Using aH/CL ratio ≥ 1.5 consistent with intensely diffuse myocardial tracer retention had a 97% sensitivity and 100% specificity with area under the curve 0.992, p<0.0001 for identifying ATTR cardiac amyloidosis. Conclusion 99mTc-PYP cardiac imaging distinguishes AL from ATTR cardiac amyloidosis and may be a simple, widely available method for identifying subjects with ATTR cardiac amyloidosis which should be studied in a larger prospective manner.

In this multicenter study, Tc 99m PYP cardiac imaging conferred a high level of sensitivity and specificity for differentiation of patients with ATTR cardiac amyloidosis (irrespective of genotype) from patients with AL cardiac amyloidosis and patients with nonamyloid heart failure with preserved ejection fraction. An H/CL ratio of 1.6 or greater was associated with worse survival among patients with ATTR cardiac amyloidosis. Among patients for whom there is a high clinical suspicion of cardiac amyloidosis, Tc 99m PYP may be of diagnostic and prognostic importance.

Background Development of noninvasive imaging modalities to quantify amyloid burden over time is an unmet clinical need. Technetium pyrophosphate (99mTc-PYP) scintigraphy is a simple and widely available radiotracer useful to differentiate transthyretin from light-chain amyloidosis in patients with advanced cardiac amyloidosis. We examined the utility of serial 99mTc-PYP scanning to quantify amyloid burden over time in TTR cardiac amyloidosis (ATTR-CA). Methods and Results Twenty subjects with ATTR-CA (10 wild type, 10 mutant) underwent serial 99mTc-PYP planar cardiac imaging. Cardiac retention was assessed both semiquantitatively (visual score 0, no uptake to 3, uptake greater than bone) and quantitatively (region of interest drawn over the heart, copied, and mirrored over the contralateral chest) to calculate a heart-to-contralateral (H/CL) ratio. Index scan mean visual score and H/CL were 3.0 ± 0.2 and 1.79 ± 0.2, respectively, and after an average 1.5 ± 0.5 years follow-up, did not differ, 3.0 ± 0.2, P = .33 and 1.76 ± 0.2, P = .44. H/CL change was minimal, 0.03 ± 0.17, did not correlate with time between scans, r = 0.19, P = .43, and was observed despite obvious clinical progression (increase in troponin ≥ 0.1 ng/mL, BNP ≥ 400 pg/mL, NYHA class, and/or death). Conclusions Serial 99mTc-PYP scanning in subjects with advanced ATTR-CA does not show significant changes over an average 1.5 years of follow-up despite obvious clinical progression.

Although SPECT myocardial perfusion imaging (MPI) provides valuable information about patients with chest pain, there is growing concern regarding its radiation burden and lengthy duration. New high-efficiency (HE) cameras and stress-first protocols both offer the potential to markedly reduce radiation. No previous study has assessed outcomes and radiation doses of patients undergoing MPI on an HE-SPECT camera using an ultra-low-dose stress-first protocol. Methods: One hundred patients presenting to the emergency department with chest pain who were candidates for stress-first MPI underwent injection of approximately 185 MBq (5 mCi) of 99m Tctetrofosmin at peak stress, followed by supine and prone imaging on an HE-SPECT camera. Same-day rest imaging was performed on patients with any abnormality on imaging after stress. Radiation effective dose was calculated from administered and residual activities. Patients were contacted 3 mo after discharge, and electronic records were accessed to evaluate the need for reevaluation for chest pain, additional imaging, or cardiac events. Results: Stress-only imaging was performed in 69 patients, for whom radiation effective dose averaged 0.99 mSv and study duration, 117 min. Radiation dose averaged 2.22 mSv over all patients. At 3 mo, 96 patients were free of major adverse cardiac events, repeat hospital chest pain evaluation, and repeat imaging or stress testing. One year after MPI and hospital discharge, all patients were living and without acute coronary syndrome. Conclusion: HE-SPECT stress-only imaging can be performed in more than two thirds of chest pain patients without a high pretest probability of a stress perfusion defect, with excellent prognosis, a radiation dose averaging 1 mSv, and a test duration of less than 2 h.