Acquired von Willebrand syndrome secondary to mitral and aortic regurgitation

The association of aortic valve stenosis with gastrointestinal bleeding was first described by Edward Heyde in 1958. Since then, there have been numerous case reports of Heyde syndrome in the medical literature worldwide.

Recently, the definition of Heyde syndrome has been updated to include the combination of aortic valve stenosis, intestinal angiodysplasia, and acquired von Willebrand factor syndrome (AVWS). However, an association between aortic or mitral regurgitation and AVWS is not well established.

The present case of a patient with endocarditis-associated severe aortic regurgitation and mitral regurgitation exhibited a clinically significant bleeding diathesis secondary to AVWS.

After surgical valve repair, the von Willebrand factor (VWF) activity spontaneously normalized.

AVWS secondary to cardiovascular diseases occurs from a selective loss of the largest multimers of VWF due to high shear forces in the blood circulation. Although it is established that stenotic valvular lesions are associated with AVWS, there have only been rare reports of regurgitant lesions leading to AVWS. We successfully treated this patient with perioperative supplementation of VWF and factor VIII.