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Background Despite new therapies, diagnosis of cardiac amyloidosis (CA) is often delayed. We recently developed a simple electrocardiographic (ECG) algorithm to suspect CA without the aid of advanced imaging modalities (Figure). Methods The aim of this study was to validate the algorithms' usefulness in clinical practice. ECG readings from patients with CA, heart failure with preserved ejection fraction (HFpEF), and hypertrophic cardiomyopathy (HCMP) were analyzed in a blinded fashion. Results 884 patients were included. Patients with pacemakers were excluded, leaving 827 ECGs (237 CA, 407 HFpEF, 183 HCMP) for final analysis. A characteristic pattern defined by the algorithm was visually perceptible in 165 ECGs (69.6%) of the amyloidosis patients vs. 114 (28%) of HFpEF vs. 22 (12.0%) of HCMP patients (p Conclusion This easy-to-use ECG algorithm has proven helpful to suspect CA. Our tool may significantly improve the treatment of heart failure patients by identifying those with amyloidosis-related disease. Funding Acknowledgement Type of funding sources: None.

Background Tafamidis kinetically stabilizes the tetrameric form of transthyretin (TTR) and thus may halt disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). However, the effect of tafamidis treatment on the progression of myocardial amyloid deposition is still unclear. Methods In our explorative analysis, we aimed to investigate the treatment effect of tafamidis on myocardial amyloid deposition measured by myocardial standardized uptake value (SUV) peak and SUV retention index using quantitative single-photon emission computed tomography/computed tomography (SPECT/CT) of the thorax and to observe its association with clinical parameters. Therefore, clinical, laboratory, imaging, and SPECT/CT examinations were performed in twenty consecutive ATTR-CM patients who started treatment with tafamidis 61mg, both at baseline and at a median of 6 months. Results Main results are summarized in Table 1. In brief, we observed a significant reduction of mean myocardial SUV peak (baseline: 15.50 vs. follow-up: 11.61, p Conclusion Treatment with tafamidis in patients with ATTR-CM results in a significant reduction in myocardial amyloid deposition as measured by the SUV retention index, with a threshold of −30% distinguishing patients who respond clinically from those who do not. However, a larger patient sample is needed to verify these results. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): Pfizer Inc.

Background Tafamidis kinetically stabilizes the tetrameric form of transthyretin (TTR) and thus may halt disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). In our explorative analysis, we aimed to investigate the treatment effect on functional capacity and cardiac biomarkers as well as cardiac function and structure using echocardiography and cardiac magnetic resonance imaging (CMR), and to compare patients treated with tafamidis with an untreated control cohort. Methods Consecutive ATTR-CM patients either received tafamidis 61mg (n=64) or tafamidis 20mg (n=23) or were assigned to an untreated control cohort (n=54) reflecting the natural history of the disease. Subsequently, we performed clinical, laboratory, echocardiography and CMR follow-up at a median of 9 to 12.5 months. Results Main results are summarized in Table 1. In brief, we observed evidence of improvement in functional capacity as measured by the 6-minute walk distance (6MWD) in tafamidis 61mg treated patients (baseline: 377.1m vs. follow-up: 383.2m, p=0.678) compared to a significant decline in mean 6MWD in untreated patients (388.1m vs. 336.4m, p=0.002; cohort comparison: p=0.005). Analysis of cardiac biomarkers revealed evidence of therapeutic response by a decrease in median NT-proBNP levels in patients treated with tafamidis 61mg (2633.0pg/mL vs. 2244.0pg/mL, p=0.366), whereas a significant increase was observed in untreated patients (2798.0pg/mL vs. 3422.0pg/mL, p Conclusion Treatment with tafamidis in patients with ATTR-CM results in significant improvements in functional capacity and cardiac biomarkers, and shows marked benefits in functional as well as structural imaging parameters compared with an untreated control cohort. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): Pfizer Inc.