Clin Res Cardiol (2022). https://doi.org/10.1007/s00392-022-02002-5 |
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Assessment of the therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging | ||
B. Chamling1, M. Bietenbeck2, D. Korthals3, S. Drakos2, V. Vehof2, P. Stalling2, M. Weil2, C. Meier2, A. Yilmaz2 | ||
1Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Muenster; 2Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Münster; 3Klinik für Kardiologie II - Rhythmologie, Universitätsklinikum Münster, Münster; | ||
Aims: Tafamidis was approved in Europe for the treatment of cardiomyopathy (CM) in patients with transthyretin amyloidosis (ATTR) in April 2020. So far, real-world data addressing the therapeutic value of tafamidis for the treatment of ATTR-CM are scarce. The purpose of this study was to carefully analyse the therapeutic benefit of tafamidis in patients with wild-type ATTR (ATTRwt) and CM (ATTRwt-CM) after one year of therapy based on serial multi-parametric cardiovascular magnetic resonance (CMR) imaging.
Methods: The present study comprised N=32 patients with ATTRwt-CM who underwent two serial multi-parametric CMR studies within a follow-up period of 12 ± 3 months. Baseline (BL) clinical parameters, serum biomarkers and CMR findings were compared to follow-up (FU) values in patients with treated with tafamidis 61mg daily (N=16, group A) and those without tafamidis therapy (N=16, group B). CMR studies were performed on a 1.5-T system and comprised (amongst others) cine-imaging for assessment of cardiac anatomy and function including 3D longitudinal strain assessment. In addition, a modified Look-Locker inversion recovery (MOLLI) T1-mapping sequence was performed for measurement of pre- and post-contrast myocardial T1-values with additional calculation of extracellular volume fraction (ECV)-values.
Results: Analyses of CMR data at FU compared to BL revealed some minor, however, significant differences between both groups: While left ventricular ejection fraction (LV-EF) remained unchanged in the tafamidis group A, a slight reduction in LV-EF was observed in the untreated group B (∆p=0.014). Accordingly, left ventricular mass index (LVMi) as well as left ventricular wall thickness (LVWT) remained rather constant in the treated group A, while a subtle increase in LVMi (∆p=0.014) and LVWT (∆p=0.006) was observed in the untreated group B. Regarding myocardial mapping analyses, a subtle increase in native T1- and ECV-values was observed in the untreated group B compared to the treated group A during FU (∆p=0.034 and ∆p=0.040, respectively). Based on 3D segmental longitudinal strain analyses, a worsening in apical / (basal + mid) strain ratio (reflecting worsened apical sparing) was only observed in the untreated group B (∆p=0.035). Serum NT-proBNP levels showed an overall increase in both the groups, however, the untreated group B showed a higher increase compared to the treated group [∆NT-proBNP: +144 pg/ml vs. +1154 pg/ml, ∆p=0.037]. Clinical assessment of NYHA class did not result in significant intra-group differences when BL were compared with FU, but a trend to improvement was observed in the treated group A compared to a worsening trend in group B (∆p=0.007).
Conclusion: Tafamidis does not improve cardiac phenotype in patients with ATTRwt-CM after one year of therapy. However, tafamidis seems to delay cardiac disease progression in patients with ATTRwt-CM, since patients with ATTRwt-CM but without tafamidis treatment showed a measurable cardiac disease progression already within one year of monitoring based on multi-parametric CMR imaging. |
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https://dgk.org/kongress_programme/jt2022/aV1259.html |