Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation
<p>Comparison of the distribution of total bile acid (tBA) concentrations between Fontan patients <18 and ≥18 years of age. Cholestatic patients are highlighted by the red dots (<b>A</b>). BA profiles of cholestatic and non-cholestatic Fontan patients (<b>B</b>). * <span class="html-italic">p</span> <math display="inline"><semantics> <mo>≤</mo> </semantics></math> 0.05. <b>Abbreviations:</b> See <a href="#app1-jcm-12-01240" class="html-app">Supplementary Table S1</a>.</p> "> Figure 2
<p>VWF:Ag and VWF:CB (<b>A</b>) values in cholestatic (<b>red dots</b>) and non-cholestatic Fontan patients. Comparison of VWF:Ag levels between Fontan patients with and without acetylsalicylic acid (ASA) treatment. Cholestatic patients are highlighted by the red dots (<b>B</b>). * <span class="html-italic">p</span> <math display="inline"><semantics> <mo>≤</mo> </semantics></math> 0.05. <b>Abbreviations:</b> VWF, von Willebrand factor; VWF:Ag, VWF antigen; VWF:CB, VWF collagen binding activity.</p> "> Figure 3
<p>VWF:CB/VWF:Ag ratio in cholestatic (<b>red dots</b>) and non-cholestatic Fontan patients. <b>Abbreviations:</b> VWF:Ag, von Willebrand factor antigen; VWF:CB, von Willebrand factor collagen binding activity.</p> ">
Abstract
:1. Introduction
2. Material and Methods
3. Results
3.1. Demographic Data and Clinical Characteristics
3.2. Bile Acids
3.3. Laboratory Parameters and Clinical Data
3.4. Tissue Factor Activity Analysis
3.5. Markers of Endothelial Damage
3.6. VWF Analysis
3.7. Correlation Analysis
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Non-Cholestatic FP (n = 28) | Cholestatic FP (n = 6) | p-Value | |
---|---|---|---|
Baseline demographic data | |||
Age at Fontan Procedure (y) | 3.6 (2.6–6.5) | 3.4 (3.0–4.0) | 0.973 |
Gender, female (n,%) | 13 (46.4) | 1 (16.7) | 0.179 |
Dominant ventricle: (n) | 0.383 | ||
Left | 11 | 4 | |
Right | 13 | 1 | |
Both | 4 | 1 | |
Type of Fontan: | 0.724 | ||
TCPC (lateral tunnel) | 8 | 1 | |
ECFO | 19 | 5 | |
Other (Kawashima) | 1 | 0 | |
Last follow-up | |||
Age at follow-up (y) | 23.5 (16.0–32.3) | 12.4 (9.3–28.0) | 0.183 |
Time since Fontan (y) | 18.0 (13.5–28.5) | 9.5 (6.3–24.7) | 0.191 |
Ejection fraction: (n) | 0.638 | ||
>50% | 27 (96.5%) | 6 (100%) | |
<50% | 1 (3.5%) | 0 | |
AV-valve incompetence: (n) | 0.401 | ||
Mild/moderate | 25 (89.3%) | 6 (100%) | |
Severe | 3 (10.7%) | 0 | |
Erythrocytes (×106/µL) | 5.2 (4.7–5.6) | 5.5 (5.3–5.7) | 0.175 |
Hemoglobin (g/dL) | 15.0 (13.4–15.4) | 16.4 (15.7–17.0) | 0.066 |
Hematocrit (%) | 43.5 (40.0–45.0) | 46.5 (45.0–48.7) | 0.044 |
Platelet count (×103/µL) | 196.0 (152.0–242.0) | 116.0 (70.3–217.0) | 0.013 |
ALT (U/L) | 25.5 (19.0–30.0) | 26.0 (21.8–43.0) | 0.428 |
AST (U/L) | 28.5 (22.0–36.0) | 30.5 (25.8–42.3) | 0.485 |
GGT (U/L) | 46.5 (36.5–73.8) | 99.5 (60.8–198.5) | 0.032 |
Cholinesterase (U/L) | 7241 (6263–8537) | 6187 (5814–7687) | 0.243 |
Lactate dehydrogenase (U/L) | 227.5 (175.0–255.8) | 216.5 (167.5–278.8) | 0.935 |
Bilirubin (mg/dL) | 0.8 (0.6–1.2) | 1.9 (1.2–2.9) | 0.003 |
Creatinine (mg/dL) | 0.6 (0.5–0.8) | 0.8 (0.7–0.9) | 0.219 |
Total protein (g/dL) | 7.5 (6.9–7.6) | 7.4 (7.2–7.7) | 0.785 |
Albumin (g/dL) | 4.7 (4.5–4.9) | 4.7 (4.3–4.8) | 0.629 |
NT-ProBNP (ng/mL) | 149.5 (73.8–269.3) | 338.0 (103.8–396.3) | 0.179 |
Antithrombin III (%) | 101.0 (94.0–106.5) | 85.0 (80.5–91.0) | 0.002 |
Medication: (n) | 0.204 | ||
Acetylsalicylic acid (ASA) | 16 | 1 | |
Phenprocoumone (PhC) | 11 | 4 | |
ACE inhibitor | 15 | 2 | |
Antiarrhythmic | 5 | 3 | |
PH medication | 2 | 2 |
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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, 1240. https://doi.org/10.3390/jcm12031240
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. Journal of Clinical Medicine. 2023; 12(3):1240. https://doi.org/10.3390/jcm12031240
Chicago/Turabian StyleMeinel, Katharina, Felicitas Korak, Martin Dusleag, Tanja Strini, Daniela Baumgartner, Ante Burmas, Hannes Sallmon, Barbara Zieger, Axel Schlagenhauf, and Martin Koestenberger. 2023. "Mild Acquired von Willebrand Syndrome and Cholestasis in Pediatric and Adult Patients with Fontan Circulation" Journal of Clinical Medicine 12, no. 3: 1240. https://doi.org/10.3390/jcm12031240