Libman-Sacks endocarditis and oral anticoagulation

The patient is a 34-year-old female with systemic lupus erythematosus and secondary antiphospholipid antibody syndrome, who evolved with convulsive crises, partially controlled with an anticonvulsant, and auscultation of a cardiac murmur, whose investigation showed the presence of a mitral valve vegetation. Once the diagnosis of Libman-Sacks endocarditis was established, therapy with warfarin sodium was initiated, and, after 6 months of oral anticoagulation, the patient had total control of the convulsive crises and the valvular vegetation disappeared on echocardiography. This study discusses the occurrence of Libman-Sacks endocarditis in systemic lupus erythematosus, its association with antiphospholipid antibody syndrome, and the anticoagulant therapy. A literature review is also provided.     

Mitral valve replacement in an infant with disseminated lupus erythematosus and Libman-Sacks endocarditis

The authors report a case of Libman-Sacks endocarditis of the posterior mitral leaflet in a child treated for disseminated lupus erythematosus for 8 years. Mitral valve replacement was performed. Libman-Sacks vegetations are hardly ever recognised in vivo and have never been previously reported in a child. The incidence of this cardiac complication of lupus erythematosus is probably higher than indicated in the literature. Attentive cardiac examination and systematic echocardiography should be part of routine follow-up of patients with disseminated lupus erythematosus.     

Double-valve Libman-Sacks endocarditis causing ventricular fibrillation cardiac arrest

Libman-Sacks endocarditis is a well-known and rather common cardiac manifestation of systemic lupus erythematosus. Transesophageal and transthoracic echocardiography are the definitive imaging methods used to evaluate cardiac valvular involvement in this disease. Valvular masses (vegetations) and valvular thickening are 2 common morphologic echocardiographic patterns. Libman-Sacks lesions are typically characterized by single-valve involvement and their small size of 1 to 4 mm.Herein, we present the unusual case of a 22-year-old woman with newly diagnosed systemic lupus erythematosus who had large, sterile vegetations of Libman-Sacks endocarditis that involved the mitral and aortic valves. This compromised coronary blood flow and resulted in ventricular fibrillation cardiac arrest. The vegetations were surgically excised, and the patient's cardiac function recovered. We discuss the treatment of the patient and that of Libman-Sacks endocarditis.     

Severe mitral regurgitation in Libman-Sacks endocarditis. Conservative surgery

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.     

Stenosis of a mechanical mitral prosthetic valve in a patient with systemic lupus erythematosus

A young woman with systemic lupus erythematosus (SLE) developed progressive heart failure several years after mitral valve replacement with a Bj?rk-Shiley prosthesis for treating mitral stenosis due to Libman-Sacks endocarditis. She was admitted to the hospital with pulmonary oedema. Transoesophageal echocardiography revealed stenosis of the mitral prosthesis, which was covered by fibrous tissue. Replacement of the prosthesis was done but the patient died from cerebral haemorrhage three days later. Although three cases of prosthetic valve dysfunction in SLE have been documented so far, this is to our knowledge the first report of a SLE recurrence on a tilting disc mechanical valve.     

Cardiovascular involvement in systemic lupus erythematosus: report of two cases

Cardiac abnormalities has been receiving increased attention in patients with systemic lupus erythematosus (SLE). Cardiovascular system involvement has been found to have a substantial effect on mortality and morbidity in patients with SLE [1]. Recent diagnostic methods using echocardiography examination have allowed the delineation of cardiac manifestations such as myocarditis and myocardial dysfunction, valvular disease, pericardial disease or pulmonary hypertension. A report of two cases is presented: 23-year-old man with acute myocarditis with left ventricular failure and pulmonary oedema as a initial presentation of active SLE, and 51-year-old woman with SLE, antiphospholipid antibodies, with history of cerebral embolic infarction, TIA and venous thrombosis and with mitral valvular dysfunction in course of nonbacterial thrombotic endocarditis. Pulmonary hypertension has been recognised in both patients probably as a result of vasculaopathy and intimal proliferation, vasculitis, thromboembolic disease or parenchymal lung disease in SLE. Recent advances in diagnosis and treatment have substantially improved the prognosis of patients with systemic lupus erythematosus and cardiovascular system involvement [2].     

Systemic lupus erythematosus as a cause of severe mitral regurgitation: New problem in an old disease

Clinical and morphologic observations in three patients with systemic lupus erythematosus and severe mitral regurgitation are described. Attention is called to the “healing” of Libman-Sacks endocarditis, an infrequent occurrence in patients with systemic lupus erythematosus in the era before steroid therapy. The mitral regurgitation in our patients appears to have resulted from “healing” of the Libman-Sacks vegetations by scarring and calcification. The healing is attributed to longterm corticosteroid therapy.     

Severe aortic stenosis and mitral regurgitation in a woman with systemic lupus erythematosus

Libman-Sacks endocarditis complicating systemic lupus erythematosus has rarely been reported to cause hemodynamically significant valvular lesions. This report presents a case of severe aortic stenosis combined with severe mitral regurgitation associated with systemic lupus erythematosus in a young woman who died while on the quota list for surgery.     

Mitral valve disease of systemic lupus erythematosus. A cause of severe congestive heart failure reversed by valve replacement.

Libman-Sacks endocarditis caused progressive life-threatening mitral regurgitation necessitating mitral valve replacement in an 18 year old woman with systemic lupus erythematosus (SLE). Although Libman-Sacks endocarditis is frequently seen at autopsy in patients with SLE, hemodynamically significant valvular disease due to that lesion is quite rare. We found no previous reports describing mitral regurgitation in a patient with SLE which has necessitated surgical intervention.     

Double heart valve replacement disclosing antiphospholipid syndrome]

In patients with systemic lupus erythematosus (SLE) heart valve lesions are usually discovered at echocardiography; their haemodynamic repercussions are uncommon, and valve replacement is exceptional. We report the case of a woman who had undergone aortic and mitral valve replacement before antiphospholipid antibodies were found associated with 4 ARA criteria of SLE. Histopathological examination confirmed the diagnosis of Libman-Sachs specific endocarditis. The presence of antiphospholipid antibodies leads to a discussion of their role in the physiopathology of the heart valve lesions and vascular accidents that occurred in this patient. The overlap observed between the diagnostic criteria of SLE and those of primary antiphospholipid syndrome is discussed. Heart valve lesions may be one of the modes of access to the antiphospholipid syndrome.     

Gonococcal endocarditis in a patient with systemic lupus erythematosus

There is mounting evidence that patients with systemic lupus erythematosus (SLE) are prone to disseminated neisserial infections. We describe the first proven case of gonococcal endocarditis affecting the pulmonary valve in a patient known to have SLE. The clinical clues and pitfalls in diagnosis are discussed, and the role of echocardiography is highlighted. Possible reasons for the association of gonococcal endocarditis with SLE include pre-existing Libman. Sacks endocarditis, complement deficiency and abnormalities of the reticuloendothelial system.      

Heart valve involvement in systemic lupus erythematosus: an echocardiographic study]

The purpose of this study was to evaluate the spectrum of morphologic and functional cardiac involvement in a selected population of patients with systemic lupus erythematosus (SLE) by means of echocardiography. Thirteen patients (2 male and 11 female) affected by SLE, mean age 41.9 years (range, 21-64), underwent M-Mode, two-dimensional and Doppler echocardiography. Eleven patients had renal disease and 3 of them were undergoing dialysis. One patient had findings of active disease. Six patients had systemic hypertension. None had a history suggestive of rheumatic fever or infective endocarditis. At echocardiographic study nine patients demonstrated findings of valvular involvement. These alterations were defined, according to the echocardiographic features, in two types: vegetation (verrucous Libman-Sacks endocarditis) and thickening. Vegetations were present in 6 patients, involving the mitral valve in all six and the aortic valve in three. The mitral valve vegetations were more frequent on the subannular portion of the posterior leaflet. Seven patients had valvular thickening: involvement of both mitral and aortic valve was present in five, and isolated mitral or aortic valve lesions in the remaining two patients. Combined valvular vegetation and thickening were observed in 4 patients. Eight patients had mild valvular dysfunction on Doppler examination: five isolated mitral regurgitation, two combined mitral and aortic regurgitation and one combined mitral stenosis and regurgitation. In agreement with previous reports, our study shows that valvular involvement in SLE is relatively frequent. Echocardiography can identify additional patterns of valvular lesions different from the known "verrucous Libman-Sacks endocarditis". The degree of valvular dysfunction is not important.     

Libman–Sacks endocarditis with an unusual presentation

Systemic lupus erythematosus is relatively common medical disorder with female predominance. This disorder can affect any organ system. Cardiac involvement is variable which can include pericardium, myocardium and endocardium. The endocardial involvement commonly affects mitral and aortic valves. This report discusses lupus endocarditis in young man with atypical presentation.     

Association of lupus anticoagulant with severe valvular heart disease in systemic lupus erythematosus

Two cases of systemic lupus erythematosus with hemodynamically significant mitral valve dysfunction and associated lupus anticoagulant are reported. Both patients underwent valve replacement and both had thrombus formation on the mitral valve, one pre- and the other postoperatively. Both patients suffered a number of extracardiac thromboses at different times in the course of their illness. The contribution of the lupus anticoagulant to the thrombotic problems, and its possible relationship to the pathogenesis of Libman-Sacks endocarditis are discussed.     

Rapid appearance of severe mitral regurgitation under high-dosage corticosteroid therapy in a patient with systemic lupus erythematosus

Survival of patients with systemic lupus erythematosus has increasedwith corticosteroid therapy. However, adverse effects of corticosteroidtherapy on cardiovascular structures, such as scarring and shrinkingof affected valves, are not well known. We report the case ofa 19-year-old patient who developed severe mitral insufficiencywithin a few weeks after high-dosage corticosteroid therapyhad been instituted for an acute relapse of systemic lupus erythematosus.The rapid development of severe mitral regurgitation was documentedby sequential colour Doppler echocardiography.     

Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution

Purpose

We evaluated the prevalence and progression of Libman-Sacks endocarditis in patients with systemic lupus erythematosus and any association between this valvulopathy and their clinical and laboratory characteristics.

Methods

Doppler echocardiography was performed in 342 consecutive patients with systemic lupus erythematosus (297 females and 45 males). The clinical and laboratory data were recorded. Patients were reevaluated after a follow-up period of 4 years.

Results

Libman-Sacks endocarditis was found in 38 patients (11%). In 24 of 38 patients, mitral valve involvement was found, resulting in regurgitation in all (mild in 18, moderate in 4, and severe in 2), whereas stenosis co-occurred with regurgitation in 9 patients (mild in 6 and moderate in 3). Thirteen (34%) of 38 patients had aortic valve involvement; 11 had regurgitation (mild) and 8 had stenosis (mild), coexistent with regurgitation in 6 of them. One patient had mild tricuspid regurgitation. A significant association was found between Libman-Sacks endocarditis and disease duration and activity, thromboses, stroke, thrombocytopenia, anticardiolipin antibodies, and antiphospholipid syndrome. During the follow-up period, 252 of 342 patients were reevaluated echocardiographically. Among the 38 patients with Libman-Sacks vegetations, 5 with mild mitral regurgitation at the beginning developed moderate (n = 4) and severe mitral regurgitation (n = 1), 2 patients with mitral stenosis (mild in 1 and moderate in 1) developed severe mitral regurgitation, and 2 patients with mild aortic regurgitation developed moderate and severe mitral regurgitation, whereas a significant deterioration of aortic stenosis was found. Two patients who were candidates for surgery died. Among the 213 patients without vegetations at the beginning, 8 developed new Libman-Sacks lesions.

Conclusions

Libman-Sacks vegetations can be found in approximately 1 of 10 patients with systemic lupus erythematosus, and they are associated with lupus duration, disease activity, anticardiolipin antibodies, and antiphospholipid syndrome manifestations. A progression of valve lesions may occur during long-term follow-up.     

Heart disease in systemic lupus erythematosus: diagnosis and management

Most patients suffering from systemic lupus erythematosus develop secondary heart disease at some time during the course of the primary illness. The most common forms of this type of heart disease are acute fibrinous pericarditis and hypertension. By means of echocardiography, an increased incidence of pericardial effusion has been demonstrated. Although commonly noted at autopsy, myocarditis is often clinically silent. However, endomyocardial biopsy may confirm its presence during life. Libman-Sacks endocarditis, although encountered in 40 to 50% of hearts at autopsy, is rarely diagnosed during life. When significant valve dysfunction such as aortic insufficiency or mitral regurgitation develops during the course of systemic lupus erythematosus, then Libman-Sacks endocarditis should be strongly suspected. Cardiac arrhythmias, first degree AV block, and acquired complete heart block may develop either de novo or in association with lupus pericarditis, myocarditis, vasculitis, etc. Complete congenital heart block has been reported in newborns of mothers with systemic lupus erythematosus, particularly those who have an antibody to a soluble tissue ribonucleoprotein antigen called RO(SS-A). Coronary arteritis and premature coronary atherosclerosis manifesting in either angina pectoris or myocardial infarction in young adults, particularly women suffering from systemic lupus erythematosus, have received attention recently. The development of hypertension and hyperlipidemia while such patients are receiving prolonged corticosteroid therapy has been incriminated as the significant risk factor in premature coronary atherosclerosis. Longstanding hypertension and congestive heart failure have unfavorable prognoses. This report is based on a cumulative review of 50 patients with acute and chronic systemic lupus erythematosus seen at our institution and in private practice during the last 10 years.     

Valvular heart disease and systemic lupus erythematosus: therapeutic implications

Valvular involvement is the most encountered form of heart disease in systemic lupus erythematosus (SLE). Immunoglobulin and complement deposition in the valvular structure will subsequently lead to Libman-Sacks vegetations, valve thickening, and valve regurgitation. Valvular stenosis is rarely seen. Involvement of the mitral valve is most frequently encountered. Valve disease for most patients is mild and asymptomatic, but patients in whom severe mitral regurgitation develops will present with symptoms of congestive heart failure. A heart murmur will be heard in almost all patients with moderate or severe regurgitation. Transesophageal echocardiography is the most sensitive method to detect the valvular involvement. The valvular changes, the hemodynamic status, or the symptomatology have been shown to progress, remain stable, or sometimes improve. Severe regurgitation, infective endocarditis, and thromboembolic events (mostly stroke or transitory ischemic attacks) are complications of valvular involvement in SLE. In treatment of these patients, prophylaxis of infectious endocarditis, selective antiaggregant and anticoagulant medication, and valve replacement are currently offered. The role of corticosteroid treatment is still unclear in the outcome of SLE valvulopathy.     

Establishing the Diagnosis of Libman–Sacks Endocarditis in Systemic Lupus Erythematosus

CASE  A 43-year-old female with systemic lupus erythematosus (SLE) was admitted with fever and shortness of breath 1 month after aortic valve replacement. A diagnostic workup including chemistries, complete blood count, blood cultures, chest x-ray, and 2-D echocardiogram was performed to determine the etiology of her symptoms and differentiate between acute bacterial endocarditis and Libman–Sacks endocarditis. DISCUSSION  By utilizing Duke’s criteria, antiphospholipid antibodies, and serial echocardiography, we were able to make a diagnosis of Libman–Sacks endocarditis. The patient was successfully treated for Libman–Sacks endocarditis and recovered uneventfully. CONCLUSION  This case highlights the challenges of making the correct diagnosis when 2 disease processes present with similar findings.