Nonbacterial thrombotic endocarditis (marantic endocarditis)

Patients manifest with fever, heart murmur, leukocytosis, elevated CRP, and findings of thrombotic disease.

Commonly referred to as Libman-Sacks endocarditis in the context of SLE or antiphospholipid syndrome.

Up to 50% of patients with SLE or APS have valvular abnormalities.

Prevalence on autopsy studies ranges from 0.3% to 9.3%.

This condition is characterized by sterile platelet thrombi that commonly adhere to the mitral and aortic valves.

It is most common in patients 40 years of age or older, but has been reported in neonates and children in association with congenital heart disease.

It is most often associated with malignancies, but has been reported to occur to systemic lupus, HIV, burns, tuberculosis, uremia, trauma, snakebites, and radiation exposure.

Pancreatic cancer is the most common malignancy associated with this process.

Lung cancer, colon cancer, and prostate cancer or other malignancies associated with this entity.

It is suspected that thrombophilia associated with malignant disease has a role in forming valvular lesions.

Autopsies studies suggest DIC is present in 71% of cases (lopez JA).

Valvular endothelial damage is the first step in the pathogenesis of this process and patients with rheumatic or congenital heart disease are at it increased risk.

Endothelial damage may be the result of high blood flow, trauma, immune complex deposition, complement activation, cytokine release of IL-1, IL-6, and TNF by tumor cells.

Endothelial damage results in a thrombogenic surface, which acts as a night is full platelet aggregation, fibrin deposition in formation of rugae.

Valvular lesions are usually less than 3 mm in diameter and are usually present on the atrial surface of the mitral valve or on the ventricular surface of the aortic valve.

Lesions from this entity emboli is frequently to the brain, spleen, kidney and heart.

Treatment is with anticoagulation with heparin.

Warfarin is less effective in the management of this entity, then it is unfractionated heparin or low molecular weight heparin.

Treatment of the underlying cause of the endocarditis is most beneficial.

This process is usually an indication of widespread malignancy and is associated with a poor prognosis.

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