Cardiovascular Disease |

Acquired von Willebrand Syndrome Secondary to Prosthetic Valve Thrombosis as Seen on Computed Tomography FREE TO VIEW

Jordan Ray, MD; Brian Shapiro, MD; Joseph Blackshear, MD
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Mayo Clinic, Jacksonville, FL

Chest. 2015;148(4_MeetingAbstracts):69A. doi:10.1378/chest.2280900
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SESSION TITLE: Cardiovascular Disease Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Prosthetic valve thrombosis is a serious and potentially life threatening phenomenon. The usual presentation is with acute heart failure or embolism. A high clinical suspicion and imaging, including echocardiography and Doppler, fluoroscopy, and cine Computed Topography (CT), are required to allow timely intervention.

CASE PRESENTATION: An 84 year old woman, with a history of St. Jude mechanical aortic valve replacement 13 years prior presented with syncope, effort intolerance, and melena. On admission, blood pressure was 98/40, pulse 65, with a grade II/VI systolic murmur radiating to the carotids. Her hemoglobin was 6.3g/dL, PT INR was 3.5, and INR values for the prior 3 months were 2.9, 3.5, and 3.0. After transfusion she underwent upper and lower endoscopies. Hemoglobin dropped as low as 4.6g/dL. Angiography was performed and underwent coiling of an abnormal vessel. Bleeding recurred upon re-initiation of warfarin. Double balloon enteroscopy was performed and a single bleeding point in the jejunum was treated with argon plasma coagulation. Von Willebrand factor multimer analysis revealed loss of the highest molecular weight multimers and increased low molecular weight multimer (figure 1, arrow), which with the history of bleeding defines acquired von Willebrand syndrome. Transthoracic and transesophageal echocardiography revealed a peak aortic velocity of 4.5 m/sec, but the valve leaflets were not well seen. Abnormal motion of the prosthesis was suspected by fluoroscopy. CT angiography confirmed the presence of impaired motion of one leaflet of the mechanical prosthesis (figure 2A). She underwent redo aortic prosthetic valve replacement with resolution of her dyspnea and bleeding. On replacement the etiology of the valve dysfunction was confirmed to be subacute thrombus formation (Figure 2B).

DISCUSSION: Prosthetic valve thrombosis is a serious complication following valve replacement surgery. Acquired von Willebrand syndrome may be present in 20% of patients with severe aortic stenosis. Our patient with prosthetic valve thrombosis presented with severe gastrointestinal bleeding from acquired von Willebrand syndrome due to recurrent aortic stenosis. Imaging played a key role in making the diagnosis, and the laboratory in establishing the etiology.

CONCLUSIONS: Acquired von Willebrand syndrome should be considered in patients with cardiac prostheses and new hemorrhage. Laboratory confirmation of acquired von Willebrand syndrome requires multimer analysis. Cine CT may be useful to confirm prosthetic valve thrombosis when the leaflets are not well seen on echocardiography or fluoroscopy.

Reference #1: Vincentelli, A., S. Susen, et al. (2003). "Acquired von Willebrand Syndrome in Aortic Stenosis." New England Journal of Medicine349(4): 343-349.

DISCLOSURE: The following authors have nothing to disclose: Jordan Ray, Brian Shapiro, Joseph Blackshear

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