Systematic evidence on the prevalence and clinical outcome of transthyretin amyloidosis (ATTR) is missing. We explored: (i) the prevalence of cardiac amyloidosis in various patient subgroups, (ii) survival estimates for ATTR subtypes, and (iii) the effects of novel therapeutics on the natural course of disease.

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Purpose Transcatheter aortic valve implantation (TAVI) is often followed by conduction abnormalities, leading to a permanent pacemaker implantation (PPI). Data regarding the clinical impact of PPI following TAVI is yet to be established. Methods Patients with severe and symptomatic aortic stenosis [effective orifice area (EOA) ≤1cm2] referred for TAVI at our institution were consecutively enrolled. Prospectively collected demographic, laboratory and echocardiographic data were retrospectively analyzed. Patients were stratified into two groups according to the need for PPI after TAVI and were followed up postoperatively with clinical and echocardiographic assessment. Primary clinical endpoint was all-cause mortality, as defined by the criteria proposed by the Valve Academic Research Consortium 2. Results In total, 292 patients were included (male: 50.2%, mean age: 80±7.6 years) in our study. Of these, 109 (37.5%) underwent PPI simultaneously or shortly after TAVI. The median follow-up period was 27.3 In this period, all-cause mortality showed no significant difference between patients with and those without PPI after TAVI (log-rank p=0.756), even after excluding patients with a pre-existing pacemaker from the analysis. Subgroup analysis also showed no difference in survival between patients with low ejection fraction (<50%) and those with preserved (≥50%) receiving a permanent pacemaker after TAVR (log-rank p=0.269). Taking into consideration factors that were found to associate to PPI in univariate analysis (pre TAVI - ejection fraction, pulmonary artery systolic pressure and New York Heart Association functional class) in a multivariate model, pre TAVI pulmonary artery systolic pressure was found to be an independent predictor of peri-procedural PPI [Exp(B): 0.977, 95% Confidence Interval: 0.957–0.998, B=−0.023, p=0.029]. Pre-TAVI conduction abnormalities and the degree of aortic annulus calcification, as assessed by computed-tomography, were not found to predict PPI after TAVI. Conclusion PPI following TAVI was not associated with survival at 27 months of follow-up, independently from the pre TAVI ejection fraction. Figure 1 Funding Acknowledgement Type of funding source: None

Background Electrocardiographic (ECG) strain has been linked to excess cardiovascular morbidity and mortality in asymptomatic patients with aortic stenosis. Purpose We aim to determine the differential impact of baseline ECG-strain on long-term mortality after transcatheter aortic valve implantation (TAVI). Methods Patients with severe and symptomatic aortic stenosis (effective orifice area [EOA]≤1cm2), who were scheduled for TAVI with a self-expanding valve between May 2015 and May 2018 were consecutively enrolled. Left ventricular strain was defined as the presence of ≥1mm convex ST-segment depression with asymmetrical T-wave inversion in leads V5 to V6 on baseline ECG. Patients were excluded, if they had bundle branch block or a permanent pacemaker at baseline. Baseline parameters were compared, and multivariate Cox proportional hazard regression models were generated to assess outcome difference. The primary clinical endpoint was cumulative mortality defined according to the criteria proposed by the Valve Academic Research Consortium-2. Results Of the 171 patients screened, 56 patients were excluded due to left bundle branch block or paced rhythm. In the 115 included patients (mean age: 81.4±7), 36 patients (31.3%) had strain pattern on pre-TAVI ECG. There were no differences in baseline characteristics between the two groups. During a median follow-up of 2.32 years (IQR 1.62 to 3), 11 patients (9.6%) reached the primary clinical endpoint. Patients in the strain group had higher incidence of all-cause mortality compared to patients without left ventricular strain (25% vs 2.5%, χ2=14.4, p<0.001). Kaplan-Meier survival analysis showed a significantly decreased cumulative probability of survival at 3 years in patients with LV-strain compared with patients without LV-strain (log-rank p=0.002, Figure 1). In the multivariate analysis, left ventricular strain [Exp(B): 8.952, 95% Confidence Interval (CI): 1.215–65.938, B=2.192, p=0.031] and QRS duration [Exp(B): 1.058, 95% CI: 1.022–1.095, B=0.056, p<0.001] were found to be independent predictors of all-cause mortality after TAVI. Conclusion Baseline ECG left ventricular strain was an independent predictor of long-term mortality post TAVI. Systematic strain measurements might aid in risk-stratifying patients scheduled for TAVI. Figure 1 Funding Acknowledgement Type of funding source: None