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Aortic valve replacement in von Willebrand's disease
The Journal of Thoracic and Cardiovascular Surgery
|August 1, 1978
Summary
Managing von Willebrand disease during aortic valve replacement requires cryoprecipitate infusions to maintain factor VIII levels. This approach ensures adequate clotting in patients with this bleeding disorder.
Area of Science:
- Cardiology
- Hematology
- Genetics
Background:
- Von Willebrand disease (VWD) is an inherited bleeding disorder characterized by low levels of von Willebrand factor and factor VIII, leading to abnormal platelet function.
- The clinical presentation of VWD varies, with some patients experiencing no bleeding history despite the disorder's presence.
- Diagnosis requires detailed laboratory assessment, including bleeding time, platelet adhesiveness, and specific factor assays.
Observation:
- A patient with VWD undergoing aortic valve replacement was successfully managed using cryoprecipitate infusions.
- Factor VIII levels were closely monitored throughout the perioperative period.
- This management strategy aimed to achieve and maintain factor VIII levels between 50% and 100% of normal.
Findings:
- Cryoprecipitate infusions effectively elevated factor VIII levels to the target range, facilitating adequate hemostasis.
- This method proved superior to commercial factor VIII concentrates due to unpredictable activity of the latter.
- Close monitoring of factor VIII levels was crucial for guiding treatment decisions.
Implications:
- Cryoprecipitate is a suitable and effective treatment for managing VWD in patients undergoing major surgery like aortic valve replacement.
- Maintaining adequate factor VIII levels is critical for preventing excessive bleeding in VWD patients.
- This case highlights the importance of tailored management strategies for VWD patients with complex cardiac conditions.