Endocarditis de Libman-Sacks e insuficiencia aórtica grave en un paciente con Libman-Sacks endocarditis is the most classic heart disorder associated with. Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most. Libman-Sacks endocarditis is a classic but rarely symptomatic manifestation of . Galve E, Ordi J, Candell J, Soler Soler J. Patología del corazón de origen.

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Since its early description inLibman-Sacks endocarditis has been considered the most characteristic cardiac disorder of systemic lupus erythematosus SLEalthough not the most frequent. The frequency of endocardial disorder in SLE varies according to the diagnostic technique used, although clinical diagnosis is uncommon. In the few cases where surgical valve repair is necessary, bioprosthetic valves are not recommended, since these can lead to Libman-Sacks endocarditis 3 ; however, in relation to this there is controversy in the medical literature.

We present a case of Libman-Sacks endocarditis involving the mitral valve with rapid progression to severe regurgitation treated with reparative surgery via mitral annuloplasty.

Woman, 36 years old, diagnosed with connective tissue disease, presenting incomplete clinical criteria for SLE for 8 years, small vessel vasculitis and repeated miscarriages, without previous cardiological symptoms. She also presented distal vasculitis lesions in the fingers and toes. Routine analysis revealed anemia with hemoglobin 9. Repeated testing for antiphospholipid antibodies proved negative. Electrocardiogram demonstrated sinus tachycardia without other associated anomalies.

Chest x-ray at admission showed mild cardiomegaly and bilateral basilar interstitial lung pattern. Transthoracic and transesophageal echocardiography showed mobile verrucose nodular thickenings of mm endocaritis both mitral valves indicative of Libman-Sacks endocarditis Figure 1and severe mitral regurgitation Figure 2.

Systolic size and function of both enxocarditis were preserved. LA indicates left atrium; LV, left ventricle. Endoarditis echocardiography was done 2 years earlier. Nodular thickenings, a few millimeters in size, were seen in the mitral valve and mild mitral regurgitation compatible with Libman-Sacks endocarditis.


Swcks the suspicion of intraparenchymal pulmonary hemorrhage, high-resolution computerized tomography was requested that confirmed the diagnosis images showing bilateral masses with ground glass opacity.

Following high-dose corticosteroids and intensive diuretic treatment, hemoptysis was eliminated and the marked heart failure controlled. However, the patient remained in functional grade III due to which, 4 months after first admission, the valvular heart disease was treated via extracorporeal surgery and mitral valve repair due to the high risk of bleeding in this patient with a Carpentier ring.

Macroscopic visualization of endocarditiss valve showed mm granulomas and pathological anatomy showed fragments with fibrin deposits and nonspecific focal calcification, but compatible with the clinical diagnosis.

One year later, the patient remained free of cardiological symptoms; echocardiogram showed a mild residual mitral regurgitation similar to that at postsurgery discharge. Libman-Sacks endocarditis is characterized by verrucose lesions on the valve surfaces, although they also can be found on the free margins of the valves and, in general, in any area of the atrial or ventricular endocardium; verruca histology is usually nonspecific.

Although the origin of valvular lesions in SLE is closely linked to the presence of antiphospholipid antibodies, 4 negative endocraditis results, as in our case, are described in the medical literature in other patients with SLE and Libman-Sacks endocarditis 8 or even in nonbacterial thrombotic endocarditis without underlying disease.

In our patient, echocardiography showed small multiple endocardittis verrucose lesions in the mitral valve free margins, both in transthoracic and transesophageal echocardiography Figure 1. The most frequent functional disorder is regurgitation, as in this case.

Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome

However, when the disease has progressed for only a few years, the patient is young and lupus is active, the valvular lesions are seldom sufficiently severe to induce heart failure. In our case, an echocardiographic study 2 years earlier, demonstrating Libman-Sacks vegetations and mild mitral regurgitation, makes it possible to demonstrate rapid progression to massive mitral regurgitation with congestive heart failure, due to which surgical intervention was indicated.


The treatment of the patients with lupus-associated valvulopathy includes endocarditis prophylaxis, antiaggregant or anticoagulant treatment in selected cases, and valve replacement when the valvular disorder is severe 11 ; the role of corticosteroid treatment regarding this valvulopathy remains undetermined.

Some authors suggest the superiority of mechanical prostheses in this type of disorder vs bioprotheses, including cryopreserved homografts, since the latter can lead to lupus valvulitis on the new valve.

Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome

In our case, the 2 recent episodes of massive pulmonary hemorrhage with severe hemoptysis, and the need for high doses of corticosteroids to control the disease guided the choice of reparative surgery of the valve. Although the evolution of the patient will have to be monitored in the coming years via serial echocardiographic studies, 1 year after surgery the state of the valvular repair is optimal.

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Previous Article Vol September Endocarditiis article. Abstract Libman-Sacks endocarditis is a classic but rarely symptomatic manifestation of systemic lupus erythematosus, and valvular surgery is needed in a few cases.

We present a patient with systemic lupus erythematosus and Libman-Sacks endocarditis that progressed rapidly to severe mitral regurgitation that needed surgery; surgical valve repair was decided upon. The literature on this topic is reviewed. Iberoamerican Cardiovascular Journals Editors’ Network.