Objectives and background: The aim of the study was to evaluate the long-term effects of tafamidis on cardiac disease progression beyond 12 months of treatment by performing (among others) serial multi-parametric cardiovascular magnetic resonance (CMR) studies in patients with transthyretin (ATTR) cardiac amyloidosis (CA) cardiomyopathy (ATTR-CM). Methods: Patients with confirmed ATTR-CM ( N = 56) were divided into two groups: in the larger group A ( N = 39; 95% male), treatment with tafamidis 61 mg once daily was initiated after the first CMR study, whereas group B ( N = 17; 76% male) comprised ATTR-CM patients who did not receive tafamidis. The observational follow-up period lasted 27 ± 6 months. During this period, patients underwent two multi-parametric CMR studies at our institution as part of a routine clinical observation pipeline. Results: Clinical symptoms assessed by the NYHA class showed a slight, however, significant increase in both groups. NT-proBNP levels substantially increased in both groups at follow-up, however, with a significantly higher increase in the tafamidis-naïve group B ( p = 0.014). LV systolic function, defined by LV-EF and 3D global longitudinal peak strain, significantly worsened in both groups at follow-up (54% to 48% p < 0.001 vs 56% to 46%, p < 0.001; −7.4 to −5.3, p < 0.001 vs −8.8 to −4.8, p < 0.001). However, the tafamidis-naïve group B experienced a substantially higher impairment of both parameters when compared to group A (∆p = 0.008 and ∆p = 0.003, respectively). LV wall thickness considerably increased in both groups at follow-up, however, with a significantly higher increase in the tafamidis-naïve group B (from 18.2 mm to 21.1 mm at follow-up, p < 0.001) compared to the tafamidis-treated group A (from 18.5 mm to 19.2 mm, p = 0.012; ∆ p < 0.001). Both global native T1 and global ECV values were significantly elevated in both groups—at baseline and at follow-up—with a significant increase in both groups during follow-up. However, a substantially higher increase in global ECV was observed in the tafamidis-naïve group B compared to the tafamidis-treated group A (group A: 51% to 57%, p < 0.001; group B: 50% to 67%, p < 0.001; ∆p < 0.001). Conclusion: Substantial worsening of clinical symptoms, serum biomarkers, and imaging parameters occurred in both tafamidis-treated and tafamidis-naïve ATTR-CM patients within a follow-up period of approximately 2 years. However, the “extent of worsening” is significantly lower in the tafamidis-treated compared to the tafamidis-naïve patients. Graphical

Aims The number of patients with cardiac implantable electronic devices (CIEDs) is increasing. However, there is limited experience regarding vasodilator-stress cardiovascular magnetic resonance (CMR) and resulting device artifacts on perfusion images. The aim of this study was to determine CMR image quality in patients with different CIED types for CMR-based perfusion stress testing. Methods and results A total of 156 patients with active CIEDs underwent CMR on a 1.5-Tesla scanner. Both conventional steady-state-free-precession (SSFP) and modified spoiled gradient-echo (sGE) protocols under stress and resting conditions were used to evaluate image artifacts in a 16-segment segment model of the heart. The study group comprised 39% conventional pacemaker (PM), 4% cardiac resynchronization therapy-pacemaker (CRT-P), 38% conventional implantable cardioverter-defibrillator (ICD), 6% cardiac resynchronization therapy-ICD (CRT-D), and 13% subcutaneous ICD (S-ICD) patients. PM-carriers showed only minor image artifacts in both perfusion protocols. Artifacts caused by ICDs were predominantly located in the left-ventricular (LV) inferolateral and anterior segments. S-ICDs showed the highest extent of artifacts with an anterolateral accentuation. The artifact extent was significantly reduced when sGE-based perfusion was used compared to SSFP-based sequences. 69% of the patients received a stress-perfusion protocol, and elective coronary angiography confirmed the presence of coronary stenosis in three cases. No major safety-relevant issues occurred. Conclusion Myocardial perfusion imaging by CMR is safe and feasible with moderate-to-high image quality in patients with all types of CIEDs, including non-conditional devices, ICDs, and S-ICDs. A sGE-based perfusion protocol should be preferred in patients with left-sided ICDs, CRT-Ds, or S-ICDs. Graphical abstract Central illustration: Artefact extent in CMR first-pass perfusion imaging in different CIED patients. PM pacemaker, ICD implantable cardioverter defibrillator, S-ICD subcutaneous ICD. The color scale reflects the mean artifact extent in percentage; dark blue = high artifact extent, light blue = low artifact extent