Aortic valve replacement for Libman-Sacks endocarditis

Abstract

A 24-year-old man with systemic lupus erythematosus and antiphospholipid syndrome complicated by lupus nephritis presented with acute limb ischaemia secondary to an embolus. Following embolectomy, the patient underwent a transthoracic echocardiogram which revealed a large vegetation on all three cusps of the aortic valve. The patient was taken for an urgent aortic valve replacement with a mechanical valve. Cultures of one cusp remained sterile. Histopathological examination of the remaining two cusps revealed sterile fibrin-rich thrombotic vegetations characteristic of non-bacterial thrombotic endocarditis.

Figure 1

Real-time three-dimensional transesophageal echocardiogram image of the aortic valve showing large, irregular vegetations on all three cusps.

Figure 2

(A) Grossly, the excised aortic valve cusps show pink-red, bulky vegetations adherent to their ventricular surfaces along the lines of closure, without perforations or cusp thickening (one cusp was submitted separately for culture). (B andC) Histopathological examination reveals features consistent with NBTE, including fibrin-rich thrombus with essentially no inflammatory component (upper two-thirds of images) on the surface of the cusp (lower third), with preservation of cusp architecture and no evidence of infection. (B: H&E stain; C: Verhoeff Van-Giesen stain; original magnification 40×). NBTE, non-bacterial thrombotic endocarditis.