Libman-Sacks Endocarditis Treatment & Management

Updated: Dec 23, 2014
  • Author: Xiushui (Mike) Ren, MD; Chief Editor: Richard A Lange, MD, MBA  more...
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Treatment

Approach Considerations

No specific therapy is indicated for Libman-Sacks endocarditis. Manage heart failure due to valvular dysfunction according to usual guidelines. [8] Medications may include vasodilators, beta blockers, diuretics, and digoxin. The efficacy of aspirin in preventing embolic phenomena with Libman-Sacks endocarditis is undetermined.

Patients with renal impairment and systemic lupus erythematosus are at a slightly increased risk for bone marrow depression and agranulocytosis with angiotensin-converting enzyme (ACE) inhibitors.

Transfer

Cardiac surgery in patients with systemic lupus erythematosus carries a relatively higher risk of complications. Transfer to a tertiary care facility for valve replacement may be warranted in some cases.

Diet

No special diet is required. However, patients with heart failure may 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.

Inpatient care

Hospitalization may be required for the following treatments related to Libman-Sacks endocarditis:

  • Stabilization of heart failure
  • Heparinization - If immediate anticoagulation is required (eg, thromboembolism)
  • Cardiac surgery
  • Treatment of infective endocarditis
  • Evaluation of cerebral ischemia

Outpatient care

Outpatient management issues may include the following:

  • Monitoring of anticoagulation
  • Adjustment of heart failure medication and monitoring of fluid status, renal function, and electrolytes
  • Antibiotic prophylaxis for dental work and procedures
  • Monitoring of underlying systemic lupus erythematosus disease activity with adjustments of steroids
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Pharmacologic Therapy

Corticosteroids

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 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.

Antibiotics

Antibiotic therapy 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 strongly 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.

Warfarin

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.

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Valve surgery

Valve surgery may be required for hemodynamically significant valvular dysfunction. [9, 10] 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 in valve surgery.

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Pregnancy-Related Considerations

Pregnancy raises several areas of concern relating to the treatment of complications of Libman-Sacks endocarditis and its associated conditions. The following considerations are important:

  • Anticoagulation with warfarin during the first trimester may result in fetal abnormalities
  • Warfarin therapy during the third trimester increases the risk of hemorrhage with delivery; however, alternative anticoagulants and antiplatelet agents are not without risk and may not be as efficacious; individualize case management
  • Avoid ACE inhibitors.
  • Maternal systemic lupus erythematosus with anti-Ro/SS-A (Sjögren syndrome antigen A) autoantibodies is associated with fetal heart block
  • The risk of spontaneous miscarriage is increased in patients with antiphospholipid syndrome
  • Do not administer tetracycline-based antibiotics
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Consultations

The following consultations may be advisable:

  • 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
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