von Willebrand factor abnormalities in aortic valve stenosis: Pathophysiology and impact on bleeding

Alessandra Casonato; Sandro Sponga; Elena Pontara; Maria Grazia Cattini; Cristina Basso; Gaetano Thiene; Giuseppe Cella; Viviana Daidone; Gino Gerosa; Antonio Pagnan

doi:10.1160/TH10-10-0634

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2011; 106(01): 58-66
DOI: 10.1160/TH10-10-0634

Blood Coagulation, Fibrinolysis and Cellular Haemostasis

Schattauer GmbH

von Willebrand factor abnormalities in aortic valve stenosis: Pathophysiology and impact on bleeding

Alessandra Casonato

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Sandro Sponga

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Elena Pontara

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Maria Grazia Cattini

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Cristina Basso

¹Department of Medical Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy

,

Gaetano Thiene

¹Department of Medical Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy

,

Giuseppe Cella

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Viviana Daidone

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Gino Gerosa

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

,

Antonio Pagnan

¹Department of Cardiologic, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy

› Author Affiliations

Abstract

Summary

Acquired von Willebrand syndrome (AVWS) may complicate severe aortic valve stenosis, due to a reduction in the haemostatically more efficient large von Willebrand factor (VWF) multimers. This study was designed to analyse the relevance of VWF abnormalities and haemorrhagic diathesis in severe aortic valve stenosis. Forty-one consecutive patients undergoing valve replacement were investigated: seven had minor bleeding symptoms in their recent history; 10 (24.3%) had a reduced VWF collagen binding (VWF:CB) to VWF antigen ratio, and 33 (80.5%) had a decrease in large VWF multimers. The shortage of large multimers was not associated with any accumulation of small VWF multimers (apparently ruling out any increased VWF proteolysis), nor was there any increase in VWF propeptide, which excludes a shorter VWF survival. The risk of developing VWF abnormalities was higher in patients with rheumatic valve disease than in degenerative cases (p=0.025) and in valves with <50% of residual endothelial cells (p=0.004). Bleeders differed from non-bleeders in that they had a higher mean transvalvular gradient and a more marked decrease in large VWF multimers. VWF abnormalities did not exacerbate peri-operative blood loss, however – a finding consistent with the full correction of these VWF abnormalities, seen already on the first postoperative day and persisting for up to six months after surgery. According to the data obtained in our cohort of patients VWF abnormalities are common in severe aortic stenosis, particularly in cases of rheumatic valve disease, but loss of the largest multimers does not seem to cause clinical bleeding in most patients.

Keywords

Aortic valve stenosis - von Willebrand factor - large VWF multimers - haemorrhagic diathesis - acquired von Willebrand syndrome

Full Text

References

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