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Liver stiffness as a prognostic parameter and tool for risk stratification in advanced cardiac transthyretin amyloidosis

Ihne-Schubert, Sandra Michaela LU ; Morbach, Caroline ; Goetze, Oliver ; Cejka, Vladimir ; Steinhardt, Maximilian Johannes ; Frantz, Stefan ; Einsele, Hermann ; Sommer, Claudia ; Störk, Stefan and Schubert, Torben LU , et al. (2024) In Clinical Research in Cardiology
Abstract

Background: In light of increasing therapeutic options, risk stratification of advanced cardiac transthyretin amyloidosis (ATTR-CA) is gaining clinical importance to avoid ineffective treatments. Liver stiffness as a marker of hypervolemia and hepatic congestion might predict mortality in advanced ATTR-CA and allow to identify patients at highest risk. Methods: Proven ATTR-CA patients underwent repeated vibration-controlled transient elastography (VTCE) and standardized serial workup within the local amyloidosis cohort study AmyKoS. Spearman correlation analyses and Cox regressions were performed to evaluate the prognostic value. Results: 41 patients with ATTR-CA were included with median age of 76.6 (55.1–89.1) years, of which 90.2%... (More)

Background: In light of increasing therapeutic options, risk stratification of advanced cardiac transthyretin amyloidosis (ATTR-CA) is gaining clinical importance to avoid ineffective treatments. Liver stiffness as a marker of hypervolemia and hepatic congestion might predict mortality in advanced ATTR-CA and allow to identify patients at highest risk. Methods: Proven ATTR-CA patients underwent repeated vibration-controlled transient elastography (VTCE) and standardized serial workup within the local amyloidosis cohort study AmyKoS. Spearman correlation analyses and Cox regressions were performed to evaluate the prognostic value. Results: 41 patients with ATTR-CA were included with median age of 76.6 (55.1–89.1) years, of which 90.2% were male and > 92% wild-type ATTR-CA. In total, 85 VCTE examinations were performed. Median follow-up was 43.7 (2.4–75.6) months; 26.8% of the patients died. At the first clinical evaluation, median left ventricular (LV) absolute global longitudinal strain (GLS) was 11.4 (5.2–19.0) % and median liver stiffness was 6.3 (2.4–22.9) kPa, both significantly correlated with mortality. NT-proBNP possessed statistically significant predictive power in ATTR-CA with more preserved LV function (absolute GLS ≥ 10), whereas stiffness seemed to be more discriminative for ATTR-CA with absolute GLS < 10. The use of alternative congestion surrogates such as liver vein dilation and tricuspid regurgitation peak velocity (tr-vmax) showed congruent results. Conclusion: Liver stiffness shows prognostic value regarding all-cause mortality and allows risk stratification in advanced ATTR-CA, particularly in those with markedly impaired longitudinal LV function. These results are transferable to other congestion surrogates.

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organization
publishing date
type
Contribution to journal
publication status
in press
subject
keywords
Cardiac amyloidosis, GLS, Liver stiffness, NT-proBNP, Transthyretin (ATTR) amyloidosis
in
Clinical Research in Cardiology
publisher
Steinkopff
external identifiers
  • pmid:39164508
  • scopus:85201664183
ISSN
1861-0684
DOI
10.1007/s00392-024-02513-3
language
English
LU publication?
yes
id
8d68ffe9-e20d-4ccf-8fc8-f7d3dd184a70
date added to LUP
2024-09-02 12:19:33
date last changed
2024-09-03 17:19:52
@article{8d68ffe9-e20d-4ccf-8fc8-f7d3dd184a70,
  abstract     = {{<p>Background: In light of increasing therapeutic options, risk stratification of advanced cardiac transthyretin amyloidosis (ATTR-CA) is gaining clinical importance to avoid ineffective treatments. Liver stiffness as a marker of hypervolemia and hepatic congestion might predict mortality in advanced ATTR-CA and allow to identify patients at highest risk. Methods: Proven ATTR-CA patients underwent repeated vibration-controlled transient elastography (VTCE) and standardized serial workup within the local amyloidosis cohort study AmyKoS. Spearman correlation analyses and Cox regressions were performed to evaluate the prognostic value. Results: 41 patients with ATTR-CA were included with median age of 76.6 (55.1–89.1) years, of which 90.2% were male and &gt; 92% wild-type ATTR-CA. In total, 85 VCTE examinations were performed. Median follow-up was 43.7 (2.4–75.6) months; 26.8% of the patients died. At the first clinical evaluation, median left ventricular (LV) absolute global longitudinal strain (GLS) was 11.4 (5.2–19.0) % and median liver stiffness was 6.3 (2.4–22.9) kPa, both significantly correlated with mortality. NT-proBNP possessed statistically significant predictive power in ATTR-CA with more preserved LV function (absolute GLS ≥ 10), whereas stiffness seemed to be more discriminative for ATTR-CA with absolute GLS &lt; 10. The use of alternative congestion surrogates such as liver vein dilation and tricuspid regurgitation peak velocity (tr-v<sub>max</sub>) showed congruent results. Conclusion: Liver stiffness shows prognostic value regarding all-cause mortality and allows risk stratification in advanced ATTR-CA, particularly in those with markedly impaired longitudinal LV function. These results are transferable to other congestion surrogates.</p>}},
  author       = {{Ihne-Schubert, Sandra Michaela and Morbach, Caroline and Goetze, Oliver and Cejka, Vladimir and Steinhardt, Maximilian Johannes and Frantz, Stefan and Einsele, Hermann and Sommer, Claudia and Störk, Stefan and Schubert, Torben and Geier, Andreas}},
  issn         = {{1861-0684}},
  keywords     = {{Cardiac amyloidosis; GLS; Liver stiffness; NT-proBNP; Transthyretin (ATTR) amyloidosis}},
  language     = {{eng}},
  publisher    = {{Steinkopff}},
  series       = {{Clinical Research in Cardiology}},
  title        = {{Liver stiffness as a prognostic parameter and tool for risk stratification in advanced cardiac transthyretin amyloidosis}},
  url          = {{http://dx.doi.org/10.1007/s00392-024-02513-3}},
  doi          = {{10.1007/s00392-024-02513-3}},
  year         = {{2024}},
}