eMedicine Specialties > Cardiology > Valvular Heart Disease

Libman-Sacks Endocarditis: Treatment & Medication

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

Treatment

Medical Care

  • No specific therapy is indicated for Libman-Sacks endocarditis.
  • The use of corticosteroids and/or cytotoxic agents for acute enlarging vegetations is controversial. Furthermore, steroid usage is implicated in the formation of leaflet thickening and valvular dysfunction. However, a recent prospective study of 56 patients with primary antiphospholipid syndrome observed that 17 patients (36%) developed new cardiac abnormalities at the 5-year follow-up. New appearances of cardiac involvement were significantly related to high immunoglobulin-G levels and anticardiolipin antibody titers, suggesting that lowering these titers with immune-modulating therapy may prevent the development of cardiac lesions.
  • Antibiotic prophylaxis is recommended for prophylaxis of secondary infective endocarditis during procedures precipitating bacteremia (eg, dental work, colonoscopy, rigid bronchoscopy, cystoscopy, colonic surgery). However, these guidelines are not well based on evidence in the case of Libman-Sacks valvular disease.
  • Anticoagulation with warfarin is often indicated for atrial fibrillation, mitral stenosis, mechanical heart valves, and thromboembolic events. High-dose anticoagulation is recommended for antiphospholipid syndrome. Case reports of resolution of valvular vegetations after warfarin therapy in patients with antiphospholipid syndrome suggest a role for anticoagulation in the treatment of valvular disease. However, therapeutic trials are lacking.
  • The efficacy of aspirin in preventing embolic phenomena with Libman-Sacks endocarditis is undetermined.
  • Manage heart failure due to valvular dysfunction according to usual guidelines. Medications might include vasodilators, beta-blockers, diuretics, and digoxin.
  • Patients with renal impairment and systemic lupus erythematosus are at slightly increased risk for bone marrow depression and agranulocytosis with ACE inhibitors.

Surgical Care

  • Valve replacement might be required for hemodynamically significant valvular dysfunction. Mechanical prostheses are usually implanted. The use of bioprosthetic (tissue) valves is debated because of concerns regarding recurrence of disease and early prosthesis degeneration.
  • Although numerous reports of uneventful valve replacement for Libman-Sacks endocarditis have been published, mortality rates in lupus patients have been reported to be as high as 25%.
  • Aggressive prophylaxis and treatment of perioperative thrombotic complications are required.

Consultations

  • Cardiologist for assistance with the evaluation of the nature and severity of valvular disease, management of heart failure, and evaluation for coexistent lupus cardiac pathology
  • Cardiac surgeon if valve replacement is required
  • Rheumatologist if underlying systemic lupus erythematosus is suggested
  • Infectious disease specialist if sepsis or secondary infective endocarditis is suggested
  • Neurologist for cerebrovascular complications

Diet

No special diet is required. However, patients with heart failure might need to avoid excessive sodium intake and patients receiving immunosuppression for systemic lupus erythematosus should avoid products that contain listerial organisms, such as soft cheeses.

Medication

Although no specific therapy is available for Libman-Sacks endocarditis, the following medications can be indicated for the management of valvular disease, complications, and associated conditions.

Antibiotic prophylaxis is recommended for prophylaxis of secondary infective endocarditis during procedures precipitating bacteremia. However, these guidelines are not well based on evidence in the case of Libman-Sacks valvular disease. The specific regimen depends on the nature of the valvular abnormalities, the procedure to be performed, allergies to antibiotics, and prior history of infective endocarditis. Consult guidelines, such as those provided by the American Heart Association, for specific information.

Limited trials have shown symptomatic benefit with ACE inhibitors for patients with mitral regurgitation and preserved left ventricular contractile function. A wide variety of drugs in this category can be used, including benazepril, captopril, enalapril, fosinopril, lisinopril, moexipril, quinapril, ramipril, and trandolapril. Avoid ACE inhibitors in patients with documented hypersensitivity and with a history of angioedema to ACE inhibitors, bilateral renal artery stenosis, severe hypotension, or second- or third-degree heart block in the absence of a pacemaker.

The hypotensive effects of ACE inhibitors can be enhanced when given concurrently with diuretics. Exercise caution in patients with hypovolemia, renal impairment, and hepatic disease. Patients with collagen vascular disease, such as systemic lupus erythematosus and scleroderma, are at slightly increased risk of bone marrow depression and agranulocytosis. Exercise caution in elderly patients; they may have decreased renal function. Hyperkalemia can result with coadministration of potassium-sparing diuretics and potassium supplements. Nonsteroidal anti-inflammatory drugs can potentiate renal dysfunction and reduce the antihypertensive effects of ACE inhibitors. Antacids can decrease the absorption of ACE inhibitors.

Anticoagulants

Indications for anticoagulation include atrial fibrillation, mitral stenosis, mechanical valves, thrombosis, thromboembolism, and antiphospholipid syndrome. Case reports have shown regression of valve masses with the use of anticoagulation, although data are not conclusive. For anticoagulation, warfarin is used regularly.


Warfarin (Coumadin)

Oral anticoagulant that suppresses hepatic synthesis of vitamin K–dependent clotting factors. Well absorbed orally, metabolized in liver, and excreted in urine and feces. Has a half-life of 0.5-1.5 d.
Loading regimen usually used for initiation of therapy. Titrate maintenance dose to desired blood PT and INR; desired INR depends on indication for anticoagulation, age of patient, and risks of bleeding versus thromboses. High-dose anticoagulation (INR 3-4) usually indicated for antiphospholipid syndrome and mechanical valves. Available tab sizes are 1, 2, 2.5, 5, 7.5, and 10 mg.

Adult

5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR

Pediatric

Not established

Griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate can decrease anticoagulant effects; oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac can increase anticoagulant effects

Documented hypersensitivity, severe liver or kidney disease, open wounds or GI ulcers, hemophilia, leukemia, blood dyscrasia, bacterial endocarditis, severe hypertension, eclampsia, preeclampsia

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients with protein C or S deficiency are at risk of developing skin necrosis

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].

  15. Leung WH, Wong KL, Lau CP, et al. Association between antiphospholipid antibodies and cardiac abnormalities in patients with systemic lupus erythematosus. Am J Med. Oct 1990;89(4):411-9. [Medline].

  16. Moder KG, Miller TD, Tazelaar HD. Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc. 1999;74:275-84. [Medline].

  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].

  18. Nihoyannopoulos P, Gomez PM, Joshi J, et al. Cardiac abnormalities in systemic lupus erythematosus. Association with raised anticardiolipin antibodies. Circulation. Aug 1990;82(2):369-75. [Medline].

  19. O'Neill D, Magaldi J, Dobkins D, Greco T. Dissolution of intracardiac mass lesions in the primary antiphospholipid antibody syndrome. Arch Intern Med. Feb 13 1995;155(3):325-7. [Medline].

  20. Roldan CA. Valvular disease associated with systemic illness. Cardiol Clin. Aug 1998;16(3):531-50. [Medline].

  21. Roldan CA, Shively BK, Crawford MH. An echocardiographic study of valvular heart disease associated with systemic lupus erythematosus. N Engl J Med. Nov 7 1996;335(19):1424-30. [Medline].

  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].

  23. Skyrme-Jones RA, Wardrop CA, Wiles CM, Fraser AG. Transesophageal echocardiographic demonstration of resolution of mitral vegetations after warfarin in a patient with the primary antiphospholipid syndrome. J Am Soc Echocardiogr. May-Jun 1995;8(3):251-6. [Medline].

  24. Turiel M, Sarzi-Puttini P, Peretti R, Bonizzato S, Muzzupappa S, Atzeni F, et al. Five-year follow-up by transesophageal echocardiographic studies in primary antiphospholipid syndrome. Am J Cardiol. Aug 15 2005;96(4):574-9. [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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