Fig 1.
Study population recruitment, selection, evaluations, and treatment.
Table 1.
Anti-inflammatory and Anti-thrombotic therapy in SLE patients with Libman-Sacks endocarditis and acute cerebrovascular disease.
Table 2.
Effect of medical therapy on clinical and laboratory parameters in SLE patients with Libman-Sacks endocarditis and cerebrovascular disease (N = 17).
Fig 2.
29-year- old male with Libman-Sacks endocarditis complicated with acute stroke and cognitive dysfunction.
A. This two-dimensional (2-D) TEE view demonstrates large (area of 1.5 cm2), sessile, and oval shaped Libman-Sacks vegetations on the distal portions and atrial side of the anterior (aml) and posterior (pml) mitral leaflets (arrows). B. This 3-dimensional (3-D) TEE let atrial (LA) view of the mitral valve demonstrates large (area of 3.20 cm2) and protruding vegetations on the tip to mid portions and atrial side of the entire anterior and posterior mitral leaflets (arrows). C. Associated severe mitral regurgitation is demonstrated by 3D-TEE color Doppler. D. Transcranial Doppler of the middle cerebral artery demonstrates a microembolic event within the spectral Doppler (upper arrow) and within the vessel (lower arrow). On MRI, multiple small white matter lesions totaling a lesion load of 4.3 cm3 were demonstrated. His neurocognitive dysfunction was graded as severe. After 3 months of aspirin and clopidogrel, hydroxychloroquine, prednisone, and mycophenolate mofetil, mitral valve vegetations significantly decreased in size (arrows) by 2D (E) and 3D TEE (F) to vegetation areas of 0.22 cm2 and 0.79 cm2, respectively, and mitral valve regurgitation improved to mild (G). Also, cerebromicroembolism resolved (H), his brain lesion load decreased to 2.1 cm3, and his neurocognitive dysfunction improved to moderate degree. Abbreviations: LA = left atrium, LV = left ventricle, aml = anterior mitral leaflet, pml = posterior mitral leaflet.
Fig 3.
31-year-old female with Libman-Sacks endocarditis complicated with acute stroke.
A. This 2-D TEE view demonstrates a large (area of 2.3 cm2) and tri-lobed Libman-Sacks vegetation extending from the atrial side of the mitral leaflets into the chorda tendinae (arrows). B. A second large (area of 1.1 cm2) vegetation is seen attached to the basal anterior wall of the left ventricle (arrow). C. This 3D-TEE view illustrates further the large and multilobed mitral valve vegetation extending from the atrial side of the mitral valve into the chordae tendinae as well as the anterior wall vegetation (arrows). D. Color Doppler TEE demonstrates only mild mitral regurgitation (arrow). E,F. After 5 days of intravenous heparin and methylprednisolone, follow-up 2D-TEE demonstrates resolution of the mitral valve vegetations (E) and significantly smaller anterior wall vegetation (F, arrow). Abbreviations as in previous Figure.
Fig 4.
18-year-old female with Libman-Sacks Endocarditis complicated with acute homonymous hemianopsia, confusional state, and cognitive dysfunction.
A,B. These 2-D TEE orthogonal views (A,B) demonstrate a large (area of 1.3 cm2), sessile, and oval shaped Libman-Sacks vegetation (arrows) on the atrial side of the anterior mitral leaflet (aml). Associated moderate mitral regurgitation was present. C. Transcranial Doppler of the right middle cerebral artery demonstrates 1 of 4 microemboli (arrows). D. Diffuse weighted imaging of the brain demonstrates bilateral periventricular cerebral infarcts (arrows) for a total lesion load of 13.71 cm3. Her global neurocognitive dysfunction was graded as moderate. Repeat studies after 9 weeks of warfarin, prednisone, and cyclophosphamide showed resolution of the mitral valve vegetation (E,F), mitral regurgitation, and cerebromicroembolism (G), improvement in brain lesions (H) with a decrease of brain lesion load to 7.72 cm3, and improvement in her global neurocognitive dysfunction to mild degree. Abbreviations as in previous Figure.
Fig 5.
21-year-old female with Libman-Sacks Endocarditis complicated with acute transient ischemic attack and previous stroke.
A,B. These 2D and color-Doppler TEE views demonstrate a moderate size (area 0.85 cm2), sessile, and oval shaped Libman-Sacks vegetations (arrows) on the distal portions and atrial side of the anterior (aml) and posterior (pml) mitral leaflets (A) and moderate to severe eccentric mitral regurgitation as demonstrated by a large flow convergence zone (arrows) (B). C. Transcranial Doppler of the left middle cerebral artery demonstrates 1 of 4 microemboli (arrows). D. On MRI, cerebral infarcts (arrowheads) and multiple white matter lesions (arrows) for a lesion load of 7.05 cm3 were demonstrated. Her global neurocognitive dysfunction was graded as severe. E,F. After 3 months of warfarin, hydroxychloroquine, prednisone, and mycophenolate mofetil, repeat imaging demonstrated resolution of the anterior mitral valve vegetation and reduction in size (arrow) of the posterior mitral leaflet vegetation (E), reduction of mitral regurgitation to mild degree (F), resolution of cerebromicroembolism (G), and decrease in count and size of white matter lesions (arrows in H) to a lesion load of 4.2 cm3, and improvement in her global neurocognitive dysfunction to mild degree.
Fig 6.
50 year old female with Libman-Sacks endocarditis complicated with an acute transient ischemic attack, past stroke, and seizure disorder.
A,B. These 2D and color-Doppler TEE views demonstrate moderate size (area of 0.85 cm2), sessile, and oval shaped Libman-Sacks vegetations (arrows) on the distal portions and atrial side of the anterior (aml) and posterior (pml) mitral leaflets (A) associated with moderate to severe highly eccentric mitral regurgitation (B). C. Transcranial Doppler of the middle cerebral artery demonstrates 1 of 2 microemboli (arrows). D. MRI of the brain demonstrates 3 cerebral infarcts (arrowheads) and multiple small periventricular and deep white matter abnormalities (arrows) for a lesion load of 1.2 cm3. Her global neurocognitive dysfunction was graded as moderate. E,F. After 5 months of warfarin, aspirin, hydroxychloroquine, and steroids, repeat TEE demonstrated significantly smaller (area 0.13 cm2) and homogeneously hyperreflectant indicative of healed mitral valve vegetations (E) and improved mitral regurgitation to moderate in degree (F). G,H. She also had no cerebromicroemboli (G), her number of brain lesions (H) and lesion load decreased to 0.7 cm3, and her neurocognitive dysfunction improved to mild degree.
Table 3.
Effect of medical therapy in patients with Libman-Sacks endocarditis.
Table 4.
Effect of medical therapy in patients with Libman-Sacks vegetations assessed by three-dimensional TEE.
Table 5.
Effect of medical therapy in patients with acute cerebrovascular disease.