Impact of tafamidis on myocardial function and CMR tissue characteristics in transthyretin amyloid cardiomyopathy.

Dobner, Stephan; Bernhard, Benedikt; Ninck, Lorenz; Wieser, Monika; Bakula, Adam; Wahl, Andreas; Köchli, Valentin; Spano, Giancarlo; Boscolo Berto, Martina; Elchinova, Elena; Safarkhanlo, Yasaman; Stortecky, Stefan; Schütze, Jonathan; Shiri, Isaac; Hunziker, Lukas; Gräni, Christoph (2024). Impact of tafamidis on myocardial function and CMR tissue characteristics in transthyretin amyloid cardiomyopathy. ESC Heart Failure, 11(5), pp. 2759-2768. Wiley 10.1002/ehf2.14815

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AIMS

Tafamidis improves clinical outcomes in transthyretin amyloid cardiomyopathy (ATTR-CM), yet how tafamidis affects cardiac structure and function remains poorly described. This study prospectively analysed the effect of tafamidis on 12-month longitudinal changes in cardiac structure and function by cardiac magnetic resonance (CMR) compared with the natural course of disease in an untreated historic control cohort.

METHODS AND RESULTS

ATTR-CM patients underwent CMR at tafamidis initiation and at 12 months. Untreated patients with serial CMRs served as reference to compare biventricular function, global longitudinal strain (GLS), LV mass and extracellular volume fraction (ECV). Thirty-six tafamidis-treated (n = 35; 97.1% male) and 15 untreated patients (n = 14; 93.3% male) with a mean age of 78.3 ± 6.5 and 76.9 ± 6.5, respectively, and comparable baseline characteristics were included. Tafamidis was associated with preserving biventricular function (LVEF (%): 50.5 ± 12 to 50.7 ± 11.5, P = 0.87; RVEF (%): 48.2 ± 10.4 to 48.2 ± 9.4, P = 0.99) and LV-GLS (-9.6 ± 3.2 to -9.9 ± 2.4%; P = 0.595) at 12 months, while a significantly reduced RV-function (50.8 ± 7.3 to 44.2 ± 11.6%, P = 0.028; P (change over time between groups) = 0.032) and numerically worsening LVGLS (-10.9 ± 3.3 to -9.1 ± 2.9%, P = 0.097; P (change over time between groups) = 0.048) was observed without treatment. LV mass significantly declined with tafamidis (184.7 ± 47.7 to 176.5 ± 44.3 g; P = 0.011), yet remained unchanged in untreated patients (163.8 ± 47.5 to 171.2 ± 39.7 g P = 0.356, P (change over time between groups) = 0.027). Irrespective of tafamidis, ECV and native T1-mapping did not change significantly from baseline to 12-month follow-up (P > 0.05).

CONCLUSIONS

Compared with untreated ATTR-CM patients, initiation of tafamidis preserved CMR-measured biventricular function and reduced LV mass at 12 months. ECV and native T1-mapping did not change significantly comparable to baseline in both groups.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology

UniBE Contributor:

Dobner, Stephan, Bernhard, Benedikt, Wieser, Monika, Bakula, Adam, Wahl, Andreas, Köchli, Valentin, Spano, Giancarlo, Boscolo Berto, Martina, Elchinova, Elena Georgieva, Safarkhanlo, Yasaman, Stortecky, Stefan, Schütze, Jonathan, Shiri Lord, Isaac, Hunziker Munsch, Lukas Christoph, Gräni, Christoph

Subjects:

600 Technology > 610 Medicine & health

ISSN:

2055-5822

Publisher:

Wiley

Language:

English

Submitter:

Pubmed Import

Date Deposited:

13 May 2024 12:29

Last Modified:

27 Sep 2024 00:13

Publisher DOI:

10.1002/ehf2.14815

PubMed ID:

38736040

Uncontrolled Keywords:

Cardiac amyloidosis Cardiac magnetic resonance imaging ECV Feature tracking GLS Tafamidis Transthyretin

BORIS DOI:

10.48350/196715

URI:

https://boris.unibe.ch/id/eprint/196715

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