Libmann-Sacks endocarditis (LSE), also known as verrucous endocarditis, is a form of nonbacterial thrombotic endocarditis characterized by large thrombi vegetations over the endocardial surface. It was considered the predominant form of endocarditis in systemic lupus erythematosus (SLE) until treatment with corticosteroids was introduced.
Data for Libman-Sacks endocarditis comes from several case controlled studies and cohort studies of patients with SLE. The prevalence of LSE in one prospective cohort study was estimated by transthoracic echocardiogram at around 11% 1. However, on post-mortem findings in older studies performed between 1950-1960 identified rates were as high as 35-65% of patients with SLE 2.
Most patients are asymptomatic as the lesions do not form near the closing line of the valves and hence valvular function is often not affected until later on during the course of the disease. Regurgitant valvular disease is more common than stenotic disease.
Since these patients are at an increased risk of forming thrombi, patients may also present with acute myocardial infarctions or ischemic strokes 3. These patients are also more prone to bacterial endocarditis and several case reports have been documented in literature citing diagnostic dilemmas 4.
SLE is characterized by the presence of autoantibodies, such as antiphospholipid antibodies. It has been postulated that there is selective deposition of complement and immune complexes along the endocardial surface leading to aggregation of platelets along the endocardial wall 5. The exact mechanism by which antiphospholipid antibodies are involved in the pathogenesis remains unclear.
Thransthoracic echocardiogram (TTE) had been considered the best initial test for the evaluation of LSE. However, recent studies have identified that trans-esophageal echocardiogram (TOE) may have a higher sensitivity, specificity, positive, and negative predictive value compared to TTE 6.
Further studies have demonstrated that three-dimensional echocardiography (3D-TEE) is better at characterizing valvular lesions (aortic and mitral valve lesions) 7. In these studies, 3D-TEE also detected more lesions in patients with cerebrovascular disease 7.
Currently, the role of 4D-flow MRI imaging as useful noninvasive tool for evaluating abnormal flow patterns, ventricular dimensions, stroke volume, and regional myocardial function, is being investigated. Some early studies have shown promising results as they have been able to more accurately demonstrate the above parameters compared to traditional TOE 8.
Treatment and prognosis
The management of LSE consists of managing the underlying disease process with appropriate immunosuppressant therapy. Patients may benefit from anticoagulation. Surgery or additional pharmacotherapy may be indicated in acute valvular rupture or heart failure.
History and etymology
It was first described by Emanuel Libman (1872-1946) and Benjamin Sacks (1896-1939), American physicians, in their 1924 seminal paper 9.
- 1. Roldan CA, Tolstrup K, Macias L, Qualls CR, Maynard D, Charlton G, Sibbitt WL. Libman-Sacks Endocarditis: Detection, Characterization, and Clinical Correlates by Three-Dimensional Transesophageal Echocardiography. (2015) Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography. 28 (7): 770-9. doi:10.1016/j.echo.2015.02.011 - Pubmed
- 2. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. (1996) Circulation. 93 (8): 1579-87. Pubmed
- 3. Ménard GE. Establishing the diagnosis of Libman-Sacks endocarditis in systemic lupus erythematosus. (2008) Journal of general internal medicine. 23 (6): 883-6. doi:10.1007/s11606-008-0627-8 - Pubmed
- 4. Cervera R, Font J, Paré C, Azqueta M, Pérez-Villa F, López-Soto A, Ingelmo M. Cardiac disease in systemic lupus erythematosus: prospective study of 70 patients. (1992) Annals of the rheumatic diseases. 51 (2): 156-9. Pubmed
- 5. Jain D, Halushka MK. Cardiac pathology of systemic lupus erythematosus. (2009) Journal of clinical pathology. 62 (7): 584-92. doi:10.1136/jcp.2009.064311 - Pubmed
- 6. Roldan CA, Qualls CR, Sopko KS, Sibbitt WL. Transthoracic versus transesophageal echocardiography for detection of Libman-Sacks endocarditis: a randomized controlled study. (2008) The Journal of rheumatology. 35 (2): 224-9. Pubmed
- 7. Moyssakis I, Tektonidou MG, Vasilliou VA, Samarkos M, Votteas V, Moutsopoulos HM. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. (2007) The American journal of medicine. 120 (7): 636-42. doi:10.1016/j.amjmed.2007.01.024 - Pubmed
- 8. Lin K, Lloyd-Jones DM, Li D, Liu Y, Yang J, Markl M, Carr JC. Imaging of cardiovascular complications in patients with systemic lupus erythematosus. (2015) Lupus. 24 (11): 1126-34. doi:10.1177/0961203315588577 - Pubmed
- 9. Libman E, Sacks B. A hitherto undescribed form of valvular and mural endocarditis. (1924) Archives of Internal Medicine. 33 (6): 701. doi:10.1001/archinte.1924.00110300044002