Acquired von Willebrand syndrome secondary to mitral and aortic regurgitation

• Published in: Journal of cardiac surgery Vol. 35; no. 9; pp. 2396 - 2398
• Main Authors: Kasai, Mio, Osako, Motohiko, Inaba, Yu, Yamabe, Kentaro, Aoki, Michiko
• Format: Journal Article
• Language: English
• Published: United States 01.09.2020
• Subjects: acquired von Willebrand syndrome; aortic regurgitation; Aortic Valve Insufficiency - etiology; Aortic Valve Insufficiency - surgery; Aortic Valve Stenosis - complications; Aortic Valve Stenosis - surgery; Blood Coagulation Tests; Heyde syndrome; Humans; mitral regurgitation; Mitral Valve Insufficiency - etiology; Mitral Valve Insufficiency - surgery; von Willebrand Diseases - complications; acquired von Willebrand syndrome; aortic regurgitation; mitral regurgitation; Heyde syndrome
• ISSN: 0886-0440; 1540-8191
• DOI: 10.1111/jocs.14796

Background 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. Aims 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. Materials & Methods The present case of a patient with endocarditis‐associated severe aortic regurgitation and mitral regurgitation exhibited a clinically significant bleeding diathesis secondary to AVWS. Results After surgical valve repair, the von Willebrand factor (VWF) activity spontaneously normalized. Discussion 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.