Therapeutic value of tafamidis in patients with wild-type transthyretin amyloidosis (ATTRwt) with cardiomyopathy based on cardiovascular magnetic resonance (CMR) imaging

© 2022. The Author(s)..

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:112

Enthalten in:

Clinical research in cardiology : official journal of the German Cardiac Society - 112(2023), 3 vom: 27. März, Seite 353-362

Sprache:

Englisch

Beteiligte Personen:

Chamling, Bishwas [VerfasserIn]
Bietenbeck, Michael [VerfasserIn]
Korthals, Dennis [VerfasserIn]
Drakos, Stefanos [VerfasserIn]
Vehof, Volker [VerfasserIn]
Stalling, Philipp [VerfasserIn]
Meier, Claudia [VerfasserIn]
Yilmaz, Ali [VerfasserIn]

Links:

Volltext

Themen:

8FG9H9D31J
Amyloidosis
CMR
ECV
Journal Article
Mapping
Myocardial strain
Tafamidis

Anmerkungen:

Date Completed 13.03.2023

Date Revised 25.04.2023

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1007/s00392-022-02035-w

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM341881465