Libman-Sacks Endocarditis and Embolic Cerebrovascular Disease

Abstract

Objective To determine whether Libman-Sacks endocarditis is a pathogenic factor for cerebrovascular disease (CVD) in systemic lupus erythematosus (SLE). Background A cardioembolic pathogenesis of SLE CVD manifested as 1) neuropsychiatric SLE (NPSLE) including stroke and transient ischemic attacks (TIA), 2) neurocognitive dysfunction, and 3) MRI focal brain lesions has not been established. Methods A 6-year study of 30 patients with acute NPSLE (27 women, age 38±12 years), 46 age-and-sex matched SLE controls without NPSLE (42 women, age 36±12 years), and 26 age-and-sex matched healthy controls (22 women, age 34±11 years) who underwent clinical and laboratory evaluations, TEE, carotid duplex, transcranial Doppler, neurocognitive testing, and brain MRI/MRA. NPSLE patients were re-evaluated after 4.5 months of therapy. All patients were followed clinically for a median of 52 months. Results Libman-Sacks vegetations (87%), cerebromicroembolism (27% with 2.5 times more events per hour), neurocognitive dysfunction (60%), and cerebral infarcts (47%) were more common in NPSLE than in SLE (28%, 20%, 33%, and 0%) and healthy controls (8%, 0%, 4%, and 0%, respectively) (all p≤0.009). Patients with vegetations had 3 times more cerebromicroemboli per hour, lower cerebral blood flow, more stroke/TIA and overall NPSLE events, neurocognitive dysfunction, cerebral infarcts, and brain lesion load than those without (all p≤0.01). Libman-Sacks vegetations were independent risk factors of NPSLE (OR=13.4, p<0.001), neurocognitive dysfunction (OR=8.0, p=0.01), brain lesions (OR=5.6, p=0.004), and all 3 outcomes combined (OR=7.5, p<0.001). Follow-up re-evaluations in 18 (78%) of 23 surviving NPSLE patients demonstrated improvement of vegetations, microembolism, brain perfusion, neurocognitive dysfunction, and lesion load (all p≤0.04). Finally, patients with vegetations had reduced event free survival time to stroke/TIA, cognitive disability, or death (p=0.007). Conclusion The presence of Libman-Sacks endocarditis in patients with SLE is associated with higher risk for embolic CVD. This suggests that Libman-Sacks endocarditis may be a source of cerebral emboli.

Libman-Sacks Endocarditis: Detection, Characterization, and Clinical Correlates by Three-Dimensional Transesophageal Echocardiography

“…The results of that study in 102 subjects (76 SLE patients and 26 healthy controls) using 2D-TEE have been published (8). All participants provided informed consent.…”

“…Therefore, accurate detection of Libman-Sacks endocarditis may lead to early therapy and prevention of the development or progression of its associated complications. Multiplane two-dimensional (2-D) transesophageal echocardiography (TEE) has high diagnostic value for detection of Libman-Sacks endocarditis (5,6,8,12). Three-dimensional TEE (3D-TEE) has emerged as an important complementary technique to 2D-TEE for detection and characterization of infective vegetations and valve tumors (13–16).…”

Nonbacterial Thrombotic Endocarditis

“…Compared to the lesions in infective endocarditis, NBTE vegetations are more friable and prone to systemic embolization. 7 NBTE is most notably associated with malignancy, antiphospholipid syndrome and SLE. 8,9 A large echocardiographic study revealed NBTE to be found in 19% of disseminated adenocarcinomas.…”

“…Unauthorized reproduction of this article is prohibited. 7 Clinical manifestations of embolization include focal neurological deficits, vision changes, memory loss, painful extremities, acute abdomen and flank pain.…”

Mitral Valve Perforation in Libman–Sacks Endocarditis: A Heart-Wrenching Case of Lupus

“…7 Although it characteristically has negligible hemodynamic significance, when present, it is strongly associated with cerebrovascular and peripheral arterial embolism, cognitive dysfunction, acute and chronic valvular dysfunction, superimposed infective endocarditis, and the need for high-risk valvular surgery. 2,8,9 There is scant literature regarding the diagnostic and treatment dilemma providers face when valve perforation occurs in the setting of LS endocarditis. We conducted a systematic review of the literature on valve perforation caused by LS endocarditis, in which we performed a PubMed query with the keywords BLibman Sacks^and Bperforation^as well as BLibman Sacks^and Brepair.^Abstracts were reviewed and relevant papers were selected.…”