AVWS in heart valve disease

Q: Which of the following cardiac valve conditions tends to cause Acquired von Willebrand syndrome (AVWS) - select one

A) Aortic stenosis (AS)

B) Mitral Stenosis (MS)

Answer: A

AS patients tend to develop AVWS and may experience cutaneous or mucosal bleeding. It directly correlates with the peak aortic gradient. The loss is primarily due to large multimers. 

A large proportion of AS patients develop anemia, with hemoglobin levels below 9 g/dL. Additionally, there is a high risk of developing gastrointestinal (GI) angiodysplasia due to both AVWS and decreased GI perfusion; the combination of AS and GI angiodysplasia is called Heyde syndrome. 

All such abnormalities resolve after corrective surgery.

AVWS is also reported in severe mitral regurgitation (MR).

#hematology

#cardiology

#surgical-critical-care

References:

1. Tamura T, Horiuchi H, Imai M, et al. Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. J Atheroscler Thromb 2015; 22:1115.

2. Kapila A, Chhabra L, Khanna A. Valvular aortic stenosis causing angiodysplasia and acquired von Willebrand's disease: Heyde's syndrome. BMJ Case Rep 2014; 2014.

3. Blackshear JL, Wysokinska EM, Safford RE, et al. Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. J Thromb Haemost 2014; 12:1966.