Surgical intervention with cardiopulmonary bypass in a patient with von Willebrand disease

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BACKGROUND: This article presents a clinical case of surgical intervention with cardiopulmonary bypass (CPB) in a patient with von Willebrand disease (VWD). VWD is the most common inherited bleeding disorder, significantly complicating surgical procedures due to impaired hemostasis. Surgical treatment of patients with VWD, particularly in the context of cardiovascular surgery involving CPB, requires an individualized approach. This includes the optimization of von Willebrand factor (vWF) and factor VIII (FVIII) levels, which are critical to preventing perioperative bleeding. The article describes the perioperative anesthetic management of the patient, emphasizing the importance of hemostasis monitoring, an individualized approach, and personalized dosing of replacement therapy to ensure effective treatment and minimize the risk of bleeding and thromboembolic complications. CASE PRESENTATION: A 36-year-old male (181 cm, 92 kg) with type 2 von Willebrand disease and a history of childhood cardiac surgery (atrial septal defect repair, right ventricular outflow tract reconstruction) was admitted for elective aortic valve replacement with a bioprosthesis due to severe aortic regurgitation. Echocardiography revealed residual ventricular septal defect shunting, aortic root dilation, moderate mitral regurgitation, and cusp prolapse. The patient had a significant bleeding history and laboratory evidence of low vWF antigen/activity and FVIII deficiency. Perioperative management included ultrasound-guided vascular access, balanced general anesthesia, moderate hypothermic cardiopulmonary bypass, antifibrinolytic therapy, and tailored hemostatic replacement with vWF concentrate, FVIII, cryoprecipitate, platelets, and plasma. The patient was extubated 6 h after surgery, required minimal transfusion support, and recovered without hemorrhagic or thromboembolic complications. He was discharged on postoperative day 10 in good condition. CONCLUSIONS: Proper management of the perioperative period is identified as a complex challenge for anesthesiologists, requiring a clear understanding of therapeutic mechanisms and their impact on coagulation.

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