Cargando…
Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging
OBJECTIVES: The purpose of this study was to carefully analyse the therapeutic benefit of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) and cardiomyopathy (ATTRwt-CM) after one year of therapy based on serial multi-parametric cardiovascular magnetic resonance (CMR) imaging....
Autores principales: | , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9998574/ https://www.ncbi.nlm.nih.gov/pubmed/35666277 http://dx.doi.org/10.1007/s00392-022-02035-w |
_version_ | 1784903494928433152 |
---|---|
author | Chamling, Bishwas Bietenbeck, Michael Korthals, Dennis Drakos, Stefanos Vehof, Volker Stalling, Philipp Meier, Claudia Yilmaz, Ali |
author_facet | Chamling, Bishwas Bietenbeck, Michael Korthals, Dennis Drakos, Stefanos Vehof, Volker Stalling, Philipp Meier, Claudia Yilmaz, Ali |
author_sort | Chamling, Bishwas |
collection | PubMed |
description | OBJECTIVES: The purpose of this study was to carefully analyse the therapeutic benefit of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) and cardiomyopathy (ATTRwt-CM) after one year of therapy based on serial multi-parametric cardiovascular magnetic resonance (CMR) imaging. BACKGROUND: Non-sponsored data based on multi-parametric CMR regarding the effect of tafamidis on the cardiac phenotype of patients with ATTRwt-CM are not available so far. METHODS: The present study comprised N = 40 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 treated “with” tafamidis 61 mg daily (n = 20, group A) and those “without” tafamidis therapy (n = 20, group B). CMR studies were performed on a 1.5-T system and comprised cine-imaging, pre- and post-contrast T1-mapping and additional calculation of extracellular volume fraction (ECV) values. RESULTS: While left ventricular ejection fraction (LV-EF), left ventricular mass index (LVMi), left ventricular wall thickness (LVWT), native T1- and ECV values remained unchanged in the tafamidis group A, a slight reduction in LV-EF (p = 0.003) as well as a subtle increase in LVMi (p = 0.034), in LVWT (p = 0.001), in native T1- (p = 0.038) and ECV-values (p = 0.017) were observed in the untreated group B. Serum NT-proBNP levels showed an overall increase in both groups, however, with the untreated group B showing a relatively higher increase compared to the treated group A. Assessment of NYHA class did not result in significant intra-group differences when BL were compared with FU, but a trend to improvement in the treated group A compared to a worsening trend in the untreated group B (∆p = 0.005). CONCLUSION: As expected, tafamidis does not improve cardiac phenotype in patients with ATTRwt-CM after one year of therapy. However, tafamidis seems to slow down cardiac disease progression in patients with ATTRwt-CM compared to those without tafamidis therapy based on multi-parametric CMR data already after one year of therapy. |
format | Online Article Text |
id | pubmed-9998574 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-99985742023-03-11 Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging Chamling, Bishwas Bietenbeck, Michael Korthals, Dennis Drakos, Stefanos Vehof, Volker Stalling, Philipp Meier, Claudia Yilmaz, Ali Clin Res Cardiol Original Paper OBJECTIVES: The purpose of this study was to carefully analyse the therapeutic benefit of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) and cardiomyopathy (ATTRwt-CM) after one year of therapy based on serial multi-parametric cardiovascular magnetic resonance (CMR) imaging. BACKGROUND: Non-sponsored data based on multi-parametric CMR regarding the effect of tafamidis on the cardiac phenotype of patients with ATTRwt-CM are not available so far. METHODS: The present study comprised N = 40 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 treated “with” tafamidis 61 mg daily (n = 20, group A) and those “without” tafamidis therapy (n = 20, group B). CMR studies were performed on a 1.5-T system and comprised cine-imaging, pre- and post-contrast T1-mapping and additional calculation of extracellular volume fraction (ECV) values. RESULTS: While left ventricular ejection fraction (LV-EF), left ventricular mass index (LVMi), left ventricular wall thickness (LVWT), native T1- and ECV values remained unchanged in the tafamidis group A, a slight reduction in LV-EF (p = 0.003) as well as a subtle increase in LVMi (p = 0.034), in LVWT (p = 0.001), in native T1- (p = 0.038) and ECV-values (p = 0.017) were observed in the untreated group B. Serum NT-proBNP levels showed an overall increase in both groups, however, with the untreated group B showing a relatively higher increase compared to the treated group A. Assessment of NYHA class did not result in significant intra-group differences when BL were compared with FU, but a trend to improvement in the treated group A compared to a worsening trend in the untreated group B (∆p = 0.005). CONCLUSION: As expected, tafamidis does not improve cardiac phenotype in patients with ATTRwt-CM after one year of therapy. However, tafamidis seems to slow down cardiac disease progression in patients with ATTRwt-CM compared to those without tafamidis therapy based on multi-parametric CMR data already after one year of therapy. Springer Berlin Heidelberg 2022-06-06 2023 /pmc/articles/PMC9998574/ /pubmed/35666277 http://dx.doi.org/10.1007/s00392-022-02035-w Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Original Paper Chamling, Bishwas Bietenbeck, Michael Korthals, Dennis Drakos, Stefanos Vehof, Volker Stalling, Philipp Meier, Claudia Yilmaz, Ali Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging |
title | Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging |
title_full | Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging |
title_fullStr | Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging |
title_full_unstemmed | Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging |
title_short | Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging |
title_sort | therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (attrwt) with cardiomyopathy based on cardiovascular magnetic resonance (cmr) imaging |
topic | Original Paper |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9998574/ https://www.ncbi.nlm.nih.gov/pubmed/35666277 http://dx.doi.org/10.1007/s00392-022-02035-w |
work_keys_str_mv | AT chamlingbishwas therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT bietenbeckmichael therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT korthalsdennis therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT drakosstefanos therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT vehofvolker therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT stallingphilipp therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT meierclaudia therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging AT yilmazali therapeuticvalueoftafamidisinpatientswithwildtypetransthyretinamyloidosisattrwtwithcardiomyopathybasedoncardiovascularmagneticresonancecmrimaging |