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Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Meinel, K; Korak, F; Dusleag, M; Strini, T; Baumgartner, D; Burmas, A; Sallmon, H; Zieger, B; Schlagenhauf, A; Koestenberger, M.
Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation.
J Clin Med. 2023; 12(3):
Doi: 10.3390/jcm12031240
[OPEN ACCESS]
Web of Science
PubMed
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- Führende Autor*innen der Med Uni Graz
- Köstenbauer Katharina
- Schlagenhauf Axel
- Co-Autor*innen der Med Uni Graz
- Baumgartner Daniela
- Burmas Ante
- Koestenberger Martin
- Sallmon Hannes
- Strini Tanja
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- Abstract:
- 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.
- Find related publications in this database (Keywords)
- Fontan circulation
- Fontan-associated liver disease
- cholestasis
- bile acids
- hemostasis
- thromboprophylaxis
- acquired von Willebrand syndrome