Hamostaseologie 2025; 45(S 01): S23-S24
DOI: 10.1055/s-0044-1801575
Abstracts
Topics
T-05 Crosstalks between hemostasis and other systems

Multicentric prospective case-control study on the prothrombotic state of single ventricle patients

A Callegari
1   Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children’s Hospital Zurich, Zürich, Switzerland
2   Children’s Research Centre, University Children’s Hospital Zurich, Zürich, Switzerland
,
M Albisetti
2   Children’s Research Centre, University Children’s Hospital Zurich, Zürich, Switzerland
3   Division of Hematology, University Children’s Hospital Zurich, Zürich, Switzerland
,
S Di Bernardo
4   Pediatric Cardiology, Department Woman-Mother-Child, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland, Lausanne, Switzerland
,
A Bosch
2   Children’s Research Centre, University Children’s Hospital Zurich, Zürich, Switzerland
3   Division of Hematology, University Children’s Hospital Zurich, Zürich, Switzerland
,
M Rizzi
5   Pediatric Hematology-Oncology Unit, Division of Pediatrics, Department Woman-Mother-Child, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Lausanne, Switzerland, Lausanne, Switzerland
,
O Kretschmar
1   Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children’s Hospital Zurich, Zürich, Switzerland
2   Children’s Research Centre, University Children’s Hospital Zurich, Zürich, Switzerland
,
D Quandt
1   Pediatric Cardiology, Pediatric Heart Center, Department of Surgery, University Children’s Hospital Zurich, Zürich, Switzerland
2   Children’s Research Centre, University Children’s Hospital Zurich, Zürich, Switzerland
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Introduction: Thrombotic and thromboembolic events remain challenging complications in single ventricle (SV) patients [1] [2]. A high frequency of clotting factor abnormalities leading to a prothrombotic state seems to play an important role. Whether the coagulation imbalance precedes or is consequence of univentricular palliation, its evolution during staged palliation, and relation to clinical outcome remain unknown.

Method: This is a multicentre, prospective, longitudinal, controlled cohort study with consecutive inclusion of 59 SV patients and 136 healthy controls (HC). Cases and HC had no difference for age (p=0.1). Blood samples for coagulation profile were taken prior to the surgical/interventional procedures at stage I (age 0.3+/- 0.3 months) and during the cardiac cath prior to stage II of SV palliation (age 4.5+/- 1.5 months). The clinical relevance of severely altered (defined as values below the 5th percentile in comparison to the HC) was assessed.

Results: SV patients had significantly reduced levels of protein C (PC) (p<0.001), free-protein-S (PS) (p<0.001), and antithrombin (AT) (p<0.001) compared to HC ([Fig. 1]). Coagulation factor (F) II (p<0.001), V (p<0.001), VII (p<0.001), IX (p=0.004), X (p=0.003), XI (p=0.012), and fibrinogen (p<0.001) as well as plasminogen (p<0.001) were reduced in SV patients. Von Willebrand factor (vWF) and FVIII were similar within the groups. At stage II PC (<0.001), AT (p<0.001), FII (p=0.03), FV (p<0.001), FVII (p<0.001) and plasminogen (p=0.01) remained reduced ([Fig. 2]). Patients with thrombotic/thromboembolic complications after stage I (n=16) had significantly reduced level of PC, PS, AT, FV, FXI, Plasminogen. Patients with severely reduced PS or/and AT both at stage I and II had a complicative clinical course after stage I and II and higher incidence of thrombotic/thromboembolic complications after stage II. No patients showed relevant increased bleeding prior/after surgery.

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Fig. 1  Box Plot comparing PC, PS and AT at stage I in patients and healthy controls.
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Fig. 2  Box Plot comparing PC, PS and AT at stage II in patients and healthy controls.

Conclusion: Abnormalities in coagulation profile in SV patients lead to a prothrombotic state, are severe and present directly after birth prior to any intervention in SV patients. Coagulation factors abnormalities are more frequent at stage I than at stage II, but a severe reduction in PC, AT, FV, FVII frequently persists at stage II. This dysbalanced coagulation parameters are associated with an increased thrombotic-/thromboembolic risk after stage I. The abnormalities prior to stage I persist at stage II in patients with a complicative clinical course and thrombotic-/thromboembolic complications after stage II.



Publikationsverlauf

Artikel online veröffentlicht:
13. Februar 2025

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  • References

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