Acquired von Willebrand syndrome
ICD-10 D68.0 · ICD-11 3B2Y

Treatment of Acquired von Willebrand Syndrome in Aortic Stenosis and Left Ventricular Assist Device

Clinical scenario

Acquired von Willebrand syndrome (AvWS) can arise as a direct consequence of cardiovascular mechanical disorders. In patients with aortic stenosis or a left ventricular assist device (LVAD), high shear stress across the abnormal valve or pump leads to proteolysis of high-molecular-weight vWF multimers, impairing primary haemostasis and creating a clinically significant bleeding risk.

Condition context

This protocol targets AvWS specifically in the cardiovascular disorder population — patients with aortic stenosis or an LVAD in whom the underlying haemodynamic mechanism drives ongoing vWF degradation. The approach must account for both the acute haemostatic deficit and the possibility of addressing the root cardiovascular cause.

Treatment approach (partial overview)

Management centres on haemostatic factor concentrate replacement for acute bleeding episodes. Additional agents and the strategy for definitive cardiovascular correction are part of the full protocol — sequencing, clinical decision points, and the complete set of options are not shown here.

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References

DOI: 10.5603/ahp.107038

Cardiovascular disorders (LVAD, aortic stenosis) — Shear-stress–induced proteolysis of HMW vWF.

vWF/FVIII concentrate; valve replacement/LVAD explant; tranexamic acid for mucosal bleeding.

Remission of AvWS has also been described after surgical removal of the tumor, cardiac surgery (valve replacement) or thyroxine substitution in hypothyroidism, as well as bloodletting in PV.

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