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Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation

Background: Hemodynamic alterations in Fontan patients (FP) are associated with hemostatic dysbalance and Fontan-associated liver disease. Studies of other hepatopathologies indicate an interplay between cholestasis, tissue factor (TF), and von Willebrand factor (VWF). Hence, we hypothesized a relat...

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Autores principales: Meinel, Katharina, Korak, Felicitas, Dusleag, Martin, Strini, Tanja, Baumgartner, Daniela, Burmas, Ante, Sallmon, Hannes, Zieger, Barbara, Schlagenhauf, Axel, Koestenberger, Martin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9917608/
https://www.ncbi.nlm.nih.gov/pubmed/36769887
http://dx.doi.org/10.3390/jcm12031240
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author Meinel, Katharina
Korak, Felicitas
Dusleag, Martin
Strini, Tanja
Baumgartner, Daniela
Burmas, Ante
Sallmon, Hannes
Zieger, Barbara
Schlagenhauf, Axel
Koestenberger, Martin
author_facet Meinel, Katharina
Korak, Felicitas
Dusleag, Martin
Strini, Tanja
Baumgartner, Daniela
Burmas, Ante
Sallmon, Hannes
Zieger, Barbara
Schlagenhauf, Axel
Koestenberger, Martin
author_sort Meinel, Katharina
collection PubMed
description Background: Hemodynamic alterations in Fontan patients (FP) are associated with hemostatic dysbalance and Fontan-associated liver disease. Studies of other hepatopathologies indicate an interplay between cholestasis, tissue factor (TF), and von Willebrand factor (VWF). Hence, we hypothesized a relationship between the accumulation of bile acids (BA) and these hemostatic factors in FP. Methods: We included 34 FP (Phenprocoumon n = 15, acetylsalicylic acid (ASA) n = 16). BA were assessed by mass spectrometry. TF activity and VWF antigen (VWF:Ag) were determined by chromogenic assays. VWF collagen-binding activity (VWF:CB) was assessed via ELISA. Results: Cholestasis was observed in 6/34 FP (total BA ≥ 10 µM). BA levels and TF activity did not correlate (p = 0.724). Cholestatic FP had lower platelet counts (p = 0.013) from which 5/6 FP were not treated with ASA. VWF:Ag levels were increased in 9/34 FP and significantly lower in FP receiving ASA (p = 0.044). Acquired von Willebrand syndrome (AVWS) was observed in 10/34-FP, with a higher incidence in cholestatic FP (4/6) (p = 0.048). Conclusions: Cholestasis is unexpectedly infrequent in FP and seems to be less frequent under ASA therapy. Therefore, ASA may reduce the risk of advanced liver fibrosis. FP should be screened for AVWS to avoid bleeding events, especially in cholestatic states.
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spelling pubmed-99176082023-02-11 Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation Meinel, Katharina Korak, Felicitas Dusleag, Martin Strini, Tanja Baumgartner, Daniela Burmas, Ante Sallmon, Hannes Zieger, Barbara Schlagenhauf, Axel Koestenberger, Martin J Clin Med Article Background: Hemodynamic alterations in Fontan patients (FP) are associated with hemostatic dysbalance and Fontan-associated liver disease. Studies of other hepatopathologies indicate an interplay between cholestasis, tissue factor (TF), and von Willebrand factor (VWF). Hence, we hypothesized a relationship between the accumulation of bile acids (BA) and these hemostatic factors in FP. Methods: We included 34 FP (Phenprocoumon n = 15, acetylsalicylic acid (ASA) n = 16). BA were assessed by mass spectrometry. TF activity and VWF antigen (VWF:Ag) were determined by chromogenic assays. VWF collagen-binding activity (VWF:CB) was assessed via ELISA. Results: Cholestasis was observed in 6/34 FP (total BA ≥ 10 µM). BA levels and TF activity did not correlate (p = 0.724). Cholestatic FP had lower platelet counts (p = 0.013) from which 5/6 FP were not treated with ASA. VWF:Ag levels were increased in 9/34 FP and significantly lower in FP receiving ASA (p = 0.044). Acquired von Willebrand syndrome (AVWS) was observed in 10/34-FP, with a higher incidence in cholestatic FP (4/6) (p = 0.048). Conclusions: Cholestasis is unexpectedly infrequent in FP and seems to be less frequent under ASA therapy. Therefore, ASA may reduce the risk of advanced liver fibrosis. FP should be screened for AVWS to avoid bleeding events, especially in cholestatic states. MDPI 2023-02-03 /pmc/articles/PMC9917608/ /pubmed/36769887 http://dx.doi.org/10.3390/jcm12031240 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Meinel, Katharina
Korak, Felicitas
Dusleag, Martin
Strini, Tanja
Baumgartner, Daniela
Burmas, Ante
Sallmon, Hannes
Zieger, Barbara
Schlagenhauf, Axel
Koestenberger, Martin
Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
title Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
title_full Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
title_fullStr Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
title_full_unstemmed Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
title_short Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
title_sort mild acquired von willebrand syndrome and cholestasis in pediatric and adult patients with fontan circulation
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9917608/
https://www.ncbi.nlm.nih.gov/pubmed/36769887
http://dx.doi.org/10.3390/jcm12031240
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