eMedicine Specialties > Cardiology > Valvular Heart Disease

Libman-Sacks Endocarditis: Differential Diagnoses & Workup

Author: Xiushui (Mike) Ren, MD, Clinical Cardiology Fellow, Division of Cardiology, Kanbar Cardiac Center, California Pacific Medical Center
Coauthor(s): Elyse Foster, MD, Director of Adult Echocardiography Laboratory, Assistant Professor, Department of Internal Medicine, Division of Cardiology, Moffitt Hospital, University of California at San Francisco; Elizabeth W Ryan, MBBS, Consulting Staff, Department of Medicine, Division of Cardiology, Austin and Repatriation General Centre, Australia
Contributor Information and Disclosures

Updated: Sep 16, 2008

Differential Diagnoses

Infective Endocarditis

Other Problems to Be Considered

Degenerative valvular disease
Fibroelastoma
Rheumatic valvular disease
Lambl excrescences (normal variant): These are filiform strands that originate at valve closure sites. They are thought to be normal variants, but some reports have proposed embolic potential.3

Workup

Laboratory Studies

Lab studies to test for Libman-Sacks Endocarditis should include the following:

  • Blood cultures: Infective endocarditis should be excluded.
  • Complete blood cell count
    • Neutrophilia might indicate infection.
    • Coexistent anemia can be present.
  • Antiphospholipid antibodies, including anticardiolipin antibody, lupus anticoagulant, VDRL, and Russell viper venom test
  • Coagulation profile with prothrombin time and activated partial thromboplastin time
  • Antinuclear antibody with or without antiextractable nuclear antigens or anti–beta2-glycoprotein
  • Anti-DNA antibody assay (double-stranded): A workup for systemic lupus erythematosus might be indicated.

Imaging Studies

  • Types of echocardiography
    • Whether to use transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) depends on the clinical scenario.
    • Transthoracic echocardiography is most appropriate for the initial evaluation of cardiac murmurs and quantification of left atrial volume and left ventricular volumes, mass, and contractile function.
    • One study compared TTE with TEE for the diagnosis of Libman-Sacks endocarditis.4  Using TEE as the standard, TTE demonstrated a low sensitivity (63% overall, 11% for valve vegetations), low specificity (58%), low negative predictive value (40%), and a moderate positive predictive value (78%) for detection of Libman-Sacks endocarditis. Thus, TEE should be performed when the TTE is nondiagnostic or when the pretest probability is high.
  • Results of echocardiography
    • Irregular borders, heterogeneous echodensity, and no independent motion characterize the masses (ie, verrucous vegetations) on the cardiac valves and endocardium. The masses are usually small and sessile, but they can be as large as 10 mm. The basal and mid portions of the mitral and aortic valves are involved most commonly.
    • Diffuse leaflet thickening of the mitral and aortic valves or focal leaflet thickening of the midbasal leaflet can be observed.
    • Acoustic shadowing suggesting calcification is possible but uncommon.
    • Valvular regurgitation can be seen.
    • Valvular stenosis may be present but is rare.
    • Left ventricular enlargement and/or dysfunction can be observed.
    • Coexistent cardiac complications of systemic lupus erythematosus may include pericardial effusion or thickening, left ventricular hypertrophy (due to hypertension), left ventricular dilatation, left ventricular segmental dysfunction, left ventricular global dysfunction, or elevated pulmonary artery pressure.
    • Transesophageal echocardiography offers superior resolution of cardiac valves and helps detect valvular lesions (especially left-sided valves) with greater sensitivity and specificity than transthoracic echocardiography.
  • Chest radiograph
    • Cardiomegaly and pulmonary congestion might be noted.
    • Calcified masses and valvular tissue are possible but are rare.

Other Tests

  • Cardiac catheterization
    • Perform coronary angiography if cardiac ischemia is suggested and if valve replacement surgery is indicated because systemic lupus erythematosus is associated with premature atherosclerotic coronary artery disease and coronary vasculitis. However, if large aortic lesions are present, noninvasive coronary artery evaluation (such as CT angiography) may be indicated because catheterization may cause embolization.
    • Left-sided pressure tracings, ventriculography, and aortography might yield additional information regarding valvular dysfunction and left ventricular function.
    • Right-sided heart catheterization is useful to determine pulmonary artery pressure and pulmonary vascular resistance because both cardiac and pulmonary pathology can occur with lupus. Use caution because the severity of valvular dysfunction in the presence of pulmonary disease might be overestimated.

Histologic Findings

The different stages of Libman-Sacks endocarditis have been described as active, active and healed, and healed lesions. Active verrucae consist of clumps of fibrin on and within the valvular leaflet tissue, which is focally necrotic, with plasma cells and lymphocytes. Combined active and healed lesions contain vascularized, fibrous tissue adjacent to fibrinous and necrotic areas. Healed lesions consist of dense, vascularized, fibrous tissue.

Histologic examinations of patients with lupus who undergo valve replacement often show the latter 2 stages, with the excised, dysfunctional leaflet tissue being fibrotic, retracted, and partially calcified with fibrinous deposits. Overlying thrombi have also been reported on valves examined at operation. Hematoxylin bodies can also be present.

Immunoglobulin and complement deposits have been identified subendothelially and in the core of vegetations.

More on Libman-Sacks Endocarditis

Overview: Libman-Sacks Endocarditis
Differential Diagnoses & Workup: Libman-Sacks Endocarditis
Treatment & Medication: Libman-Sacks Endocarditis
Follow-up: Libman-Sacks Endocarditis
Multimedia: Libman-Sacks Endocarditis
References
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References

  1. Libman E, Sacks B. A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med. 1924;33:701-37.

  2. Moyssakis I, Tektonidou MG, Vasilliou VA, Samarkos M, Votteas V, Moutsopoulos HM. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. Am J Med. July 2007;120:636-42. [Medline]. [Full Text].

  3. Nighoghossian N, Trouillas P, Perinetti M, Barthelet M, Ninet J, Loire R. [Lambl's excrescence: an uncommon cause of cerebral embolism]. Rev Neurol (Paris). Oct 1995;151(10):583-5. [Medline].

  4. Roldan CA, Qualls CR, Sopko KS, Sibbitt WL Jr. Transthoracic versus transesophageal echocardiography for detection of Libman-Sacks endocarditis: a randomized controlled study. J Rheumatol. February 2008;35:224-9. [Medline]. [Full Text].

  5. Aziz F, Baciewicz FA Jr. Lambl's excrescences: review and recommendations. Tex Heart Inst J. 2007;34(3):366-8. [Medline].

  6. Brenner B, Blumenfeld Z, Markiewicz W, Reisner SA. Cardiac involvement in patients with primary antiphospholipid syndrome. J Am Coll Cardiol. Oct 1991;18(4):931-6. [Medline].

  7. Bulkley BH, Roberts WC. The heart in systemic lupus erythematosus and the changes induced in it by corticosteroid therapy. A study of 36 necropsy patients. Am J Med. Feb 1975;58(2):243-64. [Medline].

  8. Cervera R, Font J, Pare C, et al. Cardiac disease in systemic lupus erythematosus: prospective study of 70 patients. Ann Rheum Dis. Feb 1992;51(2):156-9. [Medline].

  9. Dajee H, Hurley EJ, Szarnicki RJ. Cardiac valve replacement in systemic lupus erythematosus. A review. J Thorac Cardiovasc Surg. May 1983;85(5):718-26. [Medline].

  10. Fitzgerald D, Gaffney P, Dervan P, Doyle CT, Horgan J, Nelligan M. Giant Lambl's excrescence presenting as a peripheral embolus. Chest. Apr 1982;81(4):516-7. [Medline].

  11. Galve E, Candell-Riera J, Pigrau C, et al. Prevalence, morphologic types, and evolution of cardiac valvular disease in systemic lupus erythematosus. N Engl J Med. Sep 29 1988;319(13):817-23. [Medline].

  12. Gleason CB, Stoddard MF, Wagner SG, et al. A comparison of cardiac valvular involvement in the primary antiphospholipid syndrome versus anticardiolipin-negative systemic lupus erythematosus. Am Heart J. Apr 1993;125(4):1123-9. [Medline].

  13. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation. Apr 15 1996;93(8):1579-87. [Medline].

  14. Khamashta MA, Cervera R, Asherson RA, et al. Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus. Lancet. Jun 30 1990;335(8705):1541-4. [Medline].

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  17. Morin AM, Boyer AS, Nataf P, Gandjbakhch I. Mitral insufficiency caused by systemic lupus erythematosus requiring valve replacement: three case reports and a review of the literature. Thorac Cardiovasc Surg. Dec 1996;44(6):313-6. [Medline].

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  22. Roldan CA, Shively BK, Lau CC, et al. Systemic lupus erythematosus valve disease by transesophageal echocardiography and the role of antiphospholipid antibodies. J Am Coll Cardiol. Nov 1 1992;20(5):1127-34. [Medline].

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Further Reading

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Keywords

Libman-Sacks endocarditis, endocarditis, nonbacterial verrucous endocarditis, systemic lupus erythematosus, SLE, autoimmune disease, cardiac manifestations of autoimmune disease, verrucous vegetations, Libman-Sacks vegetations, heart valve abnormality, primary antiphospholipid syndrome, antiphospholipid syndrome, cardiac failure, cardiac vegetations

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Contributor Information and Disclosures

Author

Xiushui (Mike) Ren, MD, Clinical Cardiology Fellow, Division of Cardiology, Kanbar Cardiac Center, California Pacific Medical Center
Xiushui (Mike) Ren, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, and American Society of Echocardiography
Disclosure: Nothing to disclose.

Coauthor(s)

Elyse Foster, MD, Director of Adult Echocardiography Laboratory, Assistant Professor, Department of Internal Medicine, Division of Cardiology, Moffitt Hospital, University of California at San Francisco
Elyse Foster, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, and American Society of Echocardiography
Disclosure: Nothing to disclose.

Elizabeth W Ryan, MBBS, Consulting Staff, Department of Medicine, Division of Cardiology, Austin and Repatriation General Centre, Australia
Elizabeth W Ryan, MBBS is a member of the following medical societies: American College of Cardiology and American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Craig T Basson, MD, PhD, FAHA, FACC, Director, Cardiovascular Research, The New York Presbyterian Hospital; Professor, Greenberg Division of Cardiology, Department of Medicine, Weill Medical College of Cornell University
Craig T Basson, MD, PhD, FAHA, FACC is a member of the following medical societies: American College of Cardiology and American Heart Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Frank M Sheridan, MD, Cardiology, Providence Everett Medical Center
Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Patrice Delafontaine, MD, FACC, FAHA, FACP, FESC, Sidney W and Marilyn S Lassen Professor of Cardiovascular Medicine, Chief, Section of Cardiology, Director, Cardiovascular Center of Excellence, Tulane University; Professor of Physiology, Chair, Department of Medicine, Tulane University School of Medicine
Patrice Delafontaine, MD, FACC, FAHA, FACP, FESC is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American College of Cardiology, American College of Physicians, American Diabetes Association, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Medical Association, American Society for Clinical Investigation, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Endocrine Society, European Society of Cardiology, Louisiana State Medical Society, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

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