First described in 1926 by Erik von Willebrand as hereditary pseudohemophilia,¹ von Willebrand disease (vWD) is now recognized as the most common inherited bleeding disorder worldwide,² with an estimated prevalence of 25.6 per million population. Despite advances in diagnostic phenotyping and targeted therapy, perioperative management remains challenging in cardiac surgery, where cardiopulmonary bypass (CPB) introduces multiple hemostatic stressors including hemodilution, platelet dysfunction, inflammatory activation, and loss of high-molecular-weight Von Willebrand factor (vWF) multimers that may increase bleeding risk.  vWF plays a dual role in hemostasis by mediating platelet adhesion and stabilizing factor VIII (FVIII), protecting it from premature degradation. A deficiency of vWF leads to reduced FVIII levels and impairment of both primary and secondary hemostasis, an effect exacerbated under high shear conditions and during CPB.

 

Inherited vWD is traditionally classified into quantitative and qualitative variants. Type 1 represents a partial quantitative deficiency of vWF and is the most common form, whereas type 3 is characterized by severe or near-complete deficiency of vWF. Type 2 vWD includes qualitative defects of vWF function and is further subdivided into several variants (2A, 2B, 2M, and 2N) on the basis of specific functional abnormalities.

Current international guidelines (American Society of Hematology/International Society on Thrombosis and Haemostasis/National Hemophilia Foundation/World Federation of Hemophilia, 2021) provide recommendations for perioperative management, including target factor levels and replacement strategies. However, evidence specific to cardiac surgery remains limited to case reports and small series, restricting standardization of care and comparative assessment of available approaches.⁹ This scoping review aims to map the existing evidence on perioperative hemostatic management in patients with inherited vWD undergoing cardiac surgery, summarize key management patterns, and identify clinically relevant knowledge gaps.