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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic lupus erythematosis (SLE) is an autoimmune disease frequently accompanied by the presence of an antiphospholipid antibody (APA). Early referred to as the lupus anticoagulant (LAC), this APA consists of immunoglobulins that are known to interfere with coagulation tests that are phospholipid dependent. Such tests include the partial thromboplastin time (PTT), the activated clotting time (ACT) and may affect the thrombin time (TT). This challenges the cardiac surgical team and the perfusionist responsible for monitoring anticoagulation while performing cardiopulmonary bypass (CPB). A 46-year-old female with a history of SLE, severe mitral insufficiency, an anterior wall myocardial infarction, and the presence of a LAC was admitted for mitral valve surgery. Replacement of the mitral valve was accomplished successfully, utilizing CPB. Anticoagulation was managed using the Hepcon HMS PLUS, a device that calculates an individual's heparin dose response and permits assessment of the heparin concentration throughout the procedure. The patient recovered and was sent home 16 days after surgery.
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PMID:A case report of mitral valve replacement in a patient with lupus antibody syndrome. 1471 75

Isolated congenital heart block (ICHB) is frequently associated with neonatal lupus syndrome (NLS). Therefore few data are available regarding the long-term cardiac outcome of newborns with ICHB and the pathogenic mechanisms are not yet defined. In order to compare demographic features and cardiological outcome of patients with ICHB submitted to pacemaker (PM) implantation with and without NLS, forty ICHB patients were evaluated pre- and post-PM implantation, by clinical, electrocardiogram, Holter Monitoring, treadmill test, and electrophysiological study. According to the presence of antibodies to 52 and 60 kDa Ro/SSA and La/SSB proteins in mother's sera, it was found that 60% (24/40) of patients had ICHB associated to NLS (ICHB/NL+). Twenty-three of 24 ICHB/NL+ patients were asymptomatic, and 16 (67%) were female (P = 0.013). The frequency of syncope, mitral insufficiency (MI), and congestive heart failure (CHF) was similar pre-PM implantation in both ICHB/NL+ and ICHB/NL- groups (P > 0.05). After PM implantation, MI and CHF were only observed in ICHB/NL+ patients, although not statistically significant. Interestingly, 67% of ICHB/NL+ were noticed before one year of age while only one fourth of ICHB/NL- was diagnosed in this period (P = 0.024). Almost half (46%) of ICHB/NL+ patients required PMs in the first 24 months of life, whereas only one in the ICHB/NL- received a PM at the same age (P = 0.02). In ICHB patients requiring PM implantation, the antibody-mediated lesion seems to be associated with an earlier onset and a more severe heart disease, in spite of the uniform criteria for PM indication.
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PMID:Is isolated congenital heart block associated to neonatal lupus requiring pacemaker a distinct cardiac syndrome? 1512 17

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.
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PMID:Heart disease in systemic lupus erythematosus: diagnosis and management. 1522 37

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.
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PMID:[Severe mitral regurgitation in Libman-Sacks endocarditis. Conservative surgery]. 1618 23

Two-dimensional real-time, M-mode and Doppler echocardiographic measurements were made in 11 adult wolves (Canis lupus) anaesthetised with an intramuscular combination of medetomidine, ketamine, butorphanol and acepromazine followed by isoflurane in oxygen. M-mode measurements of the left ventricle, B-mode measurements of the left atrium and aorta, systolic indices, and Doppler measurements of aortic and pulmonary blood outflow, and of mitral and tricuspid blood inflow, were recorded. The values obtained were compared with those reported for dogs of similar bodyweight and body type. The diastolic measurements of the cardiac chambers and walls were similar to those reported for healthy, conscious dogs, but the use of anaesthesia probably resulted in the markedly different systolic cardiac measurements, systolic indices and Doppler blood flow velocities observed in the wolves. Mild mitral regurgitation, probably due to mitral endocardiosis, was observed in one wolf, and trivial functional mitral insufficiency was observed in five others.
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PMID:Echocardiographic and Doppler echocardiographic findings in 11 wolves (Canis lupus). 1644 38

We describe a case of 18-year-old woman followed for 3 years for systemic lupus erythematosis (SLE) complicated of a nephrectomy. Having like only factor of cardiovascular risk a balanced arterial hypertension. The patient was hospitalized because of choreo-athetosic's movement. We discovered fortuitously during this hospitalization an inferior myocardial necrosis as well as a mitral regurgitation. Coronary angiography was normal and the ventriculography showed an akinesy in the inferior territory. Biology made it possible to pose the diagnosis of antiphospholipid antibody syndrome (APS) on (SLE). We suppose that surgery started myocardial necrosis and underline through this case interest of early identification and appropriate treatment of APS as well as a narrow monitoring particularly in young patients candidates to surgery.
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PMID:[Silent myocardial infarction and antiphospholipid antibody syndrome]. 1757 29

Secondary amyloidosis (AA amyloidosis) has rarely been described in patients with systemic lupus erythematosus (SLE). We, herein, present a 56-year-old female patient, who developed AA amyloidosis following a 22-year history of SLE. She developed severe mitral regurgitation complicated with chordae tendinea rupture that led to acute congestive heart failure and went on a mitral valve replacement, where no flare symptoms of SLE were present. Three months after the operation, she presented with a nephrotic-range proteinuria, acute renal failure, and severe sepsis. She was found to have new vegetations on replaced valve and multi-organ failure caused her death. Re-evaluation of the excised mitral valve revealed AA amyloid deposition. Post-mortem biopsies from the kidney and bone marrow also revealed secondary amyloidosis.
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PMID:AA amyloidosis associated with systemic lupus erythematosus: impact on clinical course and outcome. 1768 56

The perforation of a mitral valve aneurysm is a rare disease which induces acute mitral regurgitation and is usually induced by infective endocarditis; however, in this case report, acute heart failure was caused by a perforated mitral valve aneurysm that was speculated to be due to Libman-Sacks endocarditis with systemic lupus erythematosis and secondary anti-phospholipid syndrome. Mitral valve plasty was performed and thereafter heart failure improved.
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PMID:Perforated mitral valve aneurysm associated with Libman-Sacks endocarditis. 1879 20

Libman-Sacks endocarditis of the mitral valve was first described by Libman and Sacks in 1924. Currently, the sterile verrucous vegetative lesions seen in Libman-Sacks endocarditis are regarded as a cardiac manifestation of both systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). Although typically mild and asymptomatic, complications of Libman-Sacks endocarditis may include superimposed bacterial endocarditis, thromboembolic events, and severe valvular regurgitation and/or stenosis requiring surgery. In this study we report two cases of mitral valve repair and two cases of mitral valve replacement for mitral regurgitation (MR) caused by Libman-Sacks endocarditis. In addition, we provide a systematic review of the English literature on mitral valve surgery for MR caused by Libman-Sacks endocarditis. This report shows that mitral valve repair is feasible and effective in young patients with relatively stable SLE and/or APS and only localized mitral valve abnormalities caused by Libman-Sacks endocarditis. Both clinical and echocardiographic follow-up after repair show excellent mid- and long-term results.
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PMID:Mitral valve surgery for mitral regurgitation caused by Libman-Sacks endocarditis: a report of four cases and a systematic review of the literature. 2033 96

A symptomatic young woman with newly diagnosed systemic lupus erythematosus and secondary antiphospholipid syndrome with elevated anticardiolipin antibodies presented with small nodules on both leaflets of the mitral valve and with a consecutive high-grade mitral regurgitation, as diagnosed by echocardiography. In consideration of the preoperative and intraoperative findings, and the patient's low age, a mitral valve repair with removal of the leaflet vegetations and a ring annuloplasty were performed.
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PMID:Mitral valve reconstruction in a patient with Libman-Sacks endocarditis: a case report. 2140 7


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