Cardiac Sarcoma Mimicking Libman-Sacks Endocarditis in a Patient with Systemic Lupus Erythematosus (SLE): A Case Report and Literature Review.

We present the case of a 39-year-old woman who was diagnosed with SLE and antiphospholipid antibodies 8 years ago. The chief manifestations of her disease included low-grade fever and polyarthritis. Eight months before presentation, she experienced symptoms attributed to a flare of SLE, leading to an increase in immunomodulatory treatment with no improvement. She presented to the emergency room with acute onset of dyspnea. Clubbing of her fingers and toes was noted. When questioned, she reported the onset of clubbing 5 months earlier. A CTA was performed to rule out pulmonary embolism, which was excluded, although it revealed a severely damaged mitral valve with severe insufficiency and a large mass on the valve, protruding into the left atrium. Antibiotics were started, with a working diagnosis of infectious endocarditis; however, the severe mitral valve dysfunction lead to emergency mitral valve replacement, revealing an organized thrombus. She was treated with anticoagulation, with a working diagnosis of Libman-Sacks endocarditis, with no improvement. Additional immunosuppression failed to improve her symptoms. Enlargement of the thrombotic mass and an increased gradient across the prosthetic mitral valve led to repeat surgery, culminating in a diagnosis of high-grade sarcoma within the left atrial mass. We further discuss cardiac sarcoma and describe the occurrence of clubbing in patients with sarcoma. This case highlights the importance of interdisciplinary collaboration and the need for vigilant monitoring in refractory cases, particularly when atypical presentations arise.

Presentation and management of marantic endocarditis: A case series.

Marantic endocarditis is defined as a sterile endocarditis that is rarely encountered in clinical practice. This case series illustrates five cases of marantic endocarditis. All cases were diagnosed on trans thoracic echocardiography and verified on transesophageal echocardiography. The first three cases occur in the setting of antiphospholipid syndrome; the last two occur in the setting of advanced malignancy. Two cases were treated successfully with anticoagulation, while two others required valvular surgery. One case resulted in mortality. The treatment course of these five patients mirrors certain patterns described in the literature.

Pathological Findings of Embolus Retrieved by Mechanical Thrombectomy in Cerebral Embolism with Libman-Sacks Endocarditis: A Report of Two Cases.

Libman-Sacks endocarditis is an important cause of embolic stroke in systemic lupus erythematosus, although the detailed pathogenesis of stroke remains unclear. We herein report two cases of stroke with Libman-Sacks endocarditis in which the emboli were retrieved by mechanical thrombectomy. The embolus consisted of eosinophilic homogeneous acellular structures, whereas fibrin-platelet thrombi were hardly observed in the embolus. Immunohistochemistry showed immunoglobulin deposits in the embolus, suggesting that immunological mechanisms were involved in the growth of the embolus. A pathological analysis of the embolus retrieved by mechanical thrombectomy provided useful information on the etiology, leading to optimal treatment.

Severe Hypertrophic Cardiomyopathy Associated With Hydroxychloroquine in a Young Woman With Systemic Lupus Erythematosus: A Case Report and Review of the Literature.

The use of the antimalarial drug hydroxychloroquine is a standard treatment in patients with systemic lupus erythematosus. It helps reduce disease-associated damage, prevents disease flare, and improves overall survival. The mechanism of action of hydroxychloroquine includes interference with lysosomal degradation of cells leading to the accumulation of vacuoles. Retinopathy is a well-described adverse effect of hydroxychloroquine, thus requiring screening with an ophthalmologist after prolonged use. Although rarely reported, cardiac adverse effects of hydroxychloroquine can also occur. In this report, we present a case of a 23-year-old woman with systemic lupus erythematosus on hydroxychloroquine who presented with stroke possibly due to Libman-Sacks endocarditis and was found to have severe hypertrophic cardiomyopathy on transthoracic echocardiogram.

Libman-Sacks endocarditis in a child with systemic lupus erythematosus: a case report and literature review.

Libman-Sacks endocarditis (LSE) is a cardiac condition characterized by the growth of verrucous vegetation. Although relatively rare in children, LSE is nevertheless a known cardiac manifestation of autoimmune diseases, including systemic lupus erythematosus (SLE). The mitral valve is the most commonly affected region, followed by the aortic valve, while the tricuspid and pulmonary valves are rarely affected. The management of established Libman-Sacks vegetation poses significant challenges, often necessitating surgical interventions, although surgery is not the primary treatment modality. Herein, we present the case of a 14-year-old Chinese female patient whose initial lupus manifestation included LSE, among other symptoms and signs that provided insights into the final diagnosis of SLE. After early comprehensive pharmacological treatment, tricuspid regurgitation and vegetation disappeared within 28 days without necessitating cardiac surgery, indicating that the resolution of LSE vegetation in this patient was achieved through a combination of immunosuppressive and anticoagulant therapy. These findings suggest the potential of this treatment approach as a viable model for the management of LSE in young patients.

Ischemic Stroke Revealing Libman-Sacks Endocarditis: A Case Report.

Libman-Sacks endocarditis (LSE) is a characteristic but rare feature of systemic lupus erythematosus (SLE), which can be found in association with antiphospholipid syndrome (APS), exposing the patient to an increased embolism risk, particularly the occurrence of ischemic stroke. We present a case involving a 64-year-old man who was admitted for ischemic stroke accompanied by a fever. Cardiac investigation revealed mitral vegetations along with severe mitral regurgitation. The diagnosis of Libman-Sacks endocarditis associated with SLE and APS was made after a laboratory work-up showing negative blood cultures and positive antibodies. This case underlines the importance of early diagnosis for better management of this pathology.

Embolic Phenomena of Libman-Sacks Endocarditis and Antiphospholipid Syndrome.

Patients with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS) are at high risk of developing arterial or venous thromboembolism and a state of systemic hypercoagulability. Libman-Sacks endocarditis (LSE) is a type of non-bacterial endocarditis usually seen in patients with systemic lupus erythematosus and antiphospholipid antibody syndrome. These vegetations dislodge easily and can cause profound neurological and systemic complications in the form of emboli. We describe one such case of a young woman with known SLE who presented with an acute middle cerebral artery (MCA) stroke and was found to have APS with extensive mitral valve vegetation, indicating Libman-Sacks endocarditis on echocardiography. Recognizing the increasing frequency of both APS and LSE in patients with SLE and screening patients, especially the younger population with SLE, for APS is vital. Furthermore, in those patients presenting with embolic events, echocardiography plays a key role as it can help expedite the diagnosis of LSE. Our case report also reiterates that warfarin, when compared to direct oral anticoagulants (DOAC), is superior in decreasing future embolic events.

Multiple organ dysfunction after mitral valve replacement in a patient with systemic lupus erythematosus complicated by Libman-Sacks endocarditis: a case report and literature review.

This case report describes a 47-year-old woman with systemic lupus erythematosus (SLE) complicated by Libman-Sacks endocarditis (LSE) who developed multiple organ dysfunction after mitral valve replacement surgery. The patient presented with a 5-day history of cough, sputum, and fever. Transthoracic echocardiography showed significant vegetations on the mitral valve. Biopsy was performed, and the pathological diagnosis was SLE complicated by LSE. After the mitral valve replacement surgery, the patient developed clinical manifestations of hepatic and renal dysfunction, cardiopulmonary failure, oliguria, and shock. The clinical symptoms significantly improved after administration of mechanical ventilation, continuous renal replacement therapy, plasma exchange, anti-inflammatory and anti-infection treatments, immunomodulatory and immunosuppressive therapies, and low-molecular-weight heparin anticoagulation. Multiple organ dysfunction after mitral valve replacement in patients with SLE complicated by LSE has rarely been reported. This report discusses the clinical manifestations, pathogenesis, and treatment of this severe complication. We hope the sharing of our experience in this case will provide a clinical basis for the treatment of severe multiple organ dysfunction after mitral valve replacement in patients with SLE complicated by LSE.

Heart valve disease in primary antiphospholipid syndrome.

OBJECTIVES: Antiphospholipid syndrome (APS)-associated heart valve disease (HVD) is well described. Nonetheless, limited data exist on clinical parameters associated with the course of primary APS (pAPS) patients with HVD. The goal of this study was to assess clinical features and related outcomes in patients with APS associated HVD. METHODS: In this multicentre retrospective study, we identified 33 pAPS patients with HVD (pAPS-HVD group) and compared their clinical course with 128 pAPS patients with normal heart valves on echocardiography (pAPS-control group). RESULTS: pAPS-HVD patients had more cerebrovascular events 56.3% vs 25% (p= 0.005) and livedo reticularis 24.2% vs 7.8% (p= 0.013) than pAPS-controls. Furthermore, catastrophic-APS (CAPS) (12.1% vs 2.4%, p= 0.034), recurrent thrombosis (33.3% vs 4.7%, p< 0.001), and need for advanced therapy (i.e. IVIG, plasmapheresis or rituximab) were more frequent in pAPS-HVD patients. Anti-B2GPI-IgG. [84.8% vs 63.2% (p= 0.034)], anti-cardiolipin IgG [90.9% vs. 64.8% (p= 0.005)] and triple positive aPL [75.8% vs 56.5% (p= 0.047)] were commoner in pAPS-HVD patients vs pAPS-controls. Ten of the 33 patients with pAPS-HVD underwent valve surgery which was associated with male gender, smoking, arterial limb ischaemia and livedo reticularis. CONCLUSION: pAPS-HVD patients had a more severe APS clinical course including CAPS and thrombotic events as well as with specific serology namely IgG isotype aPL antibodies and triple positivity. Our data suggest that pAPS-HVD represents a high-risk subgroup of APS patients.

Libman-Sacks Endocarditis Presenting as Acute Coronary Syndrome, Acute Heart Failure and Multiple Embolic Strokes.

Libman-Sacks endocarditis is a rare cardiovascular manifestation of systemic lupus erythematosus. It is described as sterile vegetative lesions which can damage heart valves resulting in complications such as acute coronary syndrome and heart failure and can embolize to cause cerebral and renal infarcts. We present the case of a young African American female presenting with pleuritic chest pain. She was initially admitted for acute coronary syndrome. She was later found to have severe mitral regurgitation and eventually received a transesophageal echocardiogram which confirmed the diagnosis of Libman-Sacks endocarditis. Her course was complicated with acute diastolic heart failure and several embolic strokes in the watershed anterior cerebral artery/middle cerebral artery (ACA/MCA) territories. She was started on anticoagulation and antiplatelet agents. Her underlying lupus was treated with immunosuppressive agents. This case demonstrates that a high index of suspicion for Libman-Sacks is crucial in patients with lupus if presenting with cardiovascular symptoms. Early and prompt diagnosis can prevent and lessen the many side effects associated with thromboembolism.

Systemic Lupus Erythematosus and Cardiovascular Diseases: A Systematic Review.

Systemic lupus erythematosus (SLE) is an autoimmune condition characterized by multi-organ involvement. The clinical presentation often varies from mild to moderate to severe. The cardiovascular system may also be affected, often portending a poor prognosis for patients. Although the relationship between SLE and cardiovascular disorders has been extensively explored through case reports and literature reviews, few systematic reviews explicitly focusing on this association have been conducted. In light of this, this systematic review aims to analyze the extent of the association between SLE and cardiovascular diseases (CVDs), by exploring the risk of developing CVDs, including myocardial infarction (MI), atherosclerosis, myocarditis, pericarditis and arrhythmias, in SLE patients vs. non-SLE patients. We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to perform the systematic review. A detailed search was done covering the period from March 2003 to March 2023 using three databases: PubMed, Google Scholar, and Cochrane. The PubMed search identified 597 articles, while Google Scholar and Cochrane searches yielded 559 and three articles, respectively. Of the 1159 articles retrieved, we chose eight for final consideration, after excluding papers that did not discuss the role of SLE in CVDs, papers published earlier than 2003, and papers with incomplete data. The eight studies chosen included two narrative reviews, two systematic reviews, and four observational studies. In this systematic review, SLE was proven to have a strong relationship with diverse CVDs, including rare ones scarcely discussed in the literature, such as vasculitis and aortic dissection. All eight of the final papers indicated a connection between SLE and CVDs, based on the systematic analysis of these articles, which revealed that most recent research supports a higher risk of peripheral arterial occlusive disease (PAOD), MI, pericarditis, myocarditis, and other cardiovascular disorders in individuals with SLE. These associations may have certain gray areas, as patient characteristics and comorbidities often affect the extent of illness and long-term prognosis. Larger-scale studies are required to probe this relationship further and research the etiopathogenesis involved in order to improve patient outcomes. The effects of SLE on the heart are, however, unequivocal.

A Diagnostic Pitfall of Transthoracic Echocardiography: A Case of a Missed Large Mitral Valve Thrombus in the Setting of Suspected Nonbacterial Thrombotic Endocarditis.

Transthoracic echocardiography (TTE) is frequently utilized in the initial evaluation of cardioembolic stroke. However, the diagnostic utility of TTE is often operator-dependent, and in conjunction with anatomical limitations, there is a range of sensitivities reported in the literature specifically in the evaluation of nonbacterial thrombotic endocarditis (NBTE). Thus, relying on TTE findings to rule out NBTE in the setting of cardioembolic stroke evaluation can lead to misdiagnosis in the absence of confirmatory transesophageal echocardiography (TEE). We present a case of a 67-year-old female with a past medical history of hypertension, diabetes mellitus, human immunodeficiency virus (HIV), and recurrent ischemic strokes who was referred by her neurologist for TEE. Despite an initial TTE with a bubble study showing no evidence of intra-atrial septum, left ventricular thrombus, or any valvular pathology, there remained high suspicion of a cardioembolic source due to the bi-hemispheric presentation of the patient's previous strokes. Prior electrocardiography and cardiac event monitor showed normal sinus rhythm. Her TEE revealed a large, dense thrombus measuring 1.0 x 0.8 centimeters involving the anterior mitral valve leaflet with associated moderate mitral regurgitation. The patient was placed on systemic anticoagulation and discharged home with outpatient follow-up with cardiology. Our case highlights the diagnostic pitfalls of TTE use in the evaluation of cardioembolic stroke with a particular emphasis on NBTE in addition to discussing the rationale for follow-up TEE when TTE is otherwise unrevealing.

Multivalvular involvement associated with Libman-Sacks endocarditis detected by multimodality imaging: A case report.

Libman-Sacks endocarditis accounts for 6-11 percent of systemic lupus erythematosus patients and is associated with varying degrees of valvular dysfunction, increased risk for stroke and transient ischemic attacks, and increased mortality. In previous studies, left-sided valvular Libman-Sacks vegetations were more frequently detected than right sided vegetations; reported cases of bilateral involvement is very rare. A comprehensive clinical assessment and the multimodality imaging is of utmost importance in the management of systemic lupus erythematosus. In this case report, we describe a 31-year-old female patient with uncontrolled systemic lupus erythematosus initially presented with gastrointestinal symptoms but eventually had a vegetation-like structure on the posterior leaflet of the mitral valve which was revealed during routine echocardiography. Two-dimensional/three-dimensional transthoracic and transesophageal echocardiography, cardiac magnetic resonance, and cardiac computed tomography further characterized the mitral valve vegetation and revealed an additional vegetation of the pulmonary valve. Echocardiography remains the cornerstone for the detection of Libman-Sacks vegetations. Cardiac MRI and cardiac CT are useful in characterizing lesion size and effects and may prove particularly helpful in the assessment of right-sided or multivalvular endocarditis. The presence of focal brain lesions on brain MRI prompted antithrombotic therapy.

Severe Libman-Sacks endocarditis complicating antiphospholipid syndrome: a retrospective analysis of 23 operated cases.

OBJECTIVE: Data on severe heart valve disease (HVD), including Libman-Sacks endocarditis, associated with SLE and/or APS requiring valvular surgery are scarce. We thus conducted a retrospective study, aimed at describing and clarifying clinical, laboratory, echocardiographic, histopathological and evolutional features of SLE and/or APS patients with severe associated-HVD. METHODS: An observational retrospective multicentric analysis of 23 adults with SLE and/or APS and HVD between 1996 and 2019 and available histopathological report evaluating long-term follow-up. RESULTS: Twenty-three individuals (20 females, median age 37 [range 17-76] years) were included. All had APS (thrombotic in 22, with an arterial phenotype in 15 and with catastrophic APS [CAPS] in six), and 11 (47%) had SLE. Systemic underlying disease had been diagnosed prior to HVD in 12 (52%). In 10 patients (43%), HVD was complicated by cerebral stroke prior to surgery. Twenty patients (87%) had only one pathological valve, the mitral valve in 18 patients (78%). Valvular thickening (n = 19) and valvular regurgitation (n = 19) were the most frequently reported lesions. Fifteen (62%) patients underwent mechanical valve replacement, six (26%) conservative valve repair (five were later re-operated after a median time of 1 [0-4] year), and two (9%) underwent biological valve replacement. Nine patients (39%) presented early-onset post-operative complications, including three CAPS immediately after surgery and one death. After surgery, 18 patients (78%) had normal postoperative valvular function, but almost half of the patients (43%) had post-operative neurological sequelae (median follow-up of 6 [2-20] years). CONCLUSION: Severe HVD leading to surgery was strongly associated with thrombotic APS, especially arterial phenotypes. Half of the reported patients presented cerebral stroke complicating the HVD. Valvular surgery carried a significant risk of CAPS.

Diagnostic Challenges of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a disease characterized by clinical heterogeneity with unpredictable course. Several disease endotypes have been identified, including SLE with antiphospholipid syndrome (APS). We report a case of a pregnant woman with hypertension and proteinuria, diagnosed with APS, Libman-Sacks endocarditis that led to moderate to severe mitral valve insufficiency, and SLE. We describe the diagnostic steps, evolution, and complications. This case highlights the asynchrony behavior of SLE, emphasizing the importance of a multidisciplinary approach to an early diagnosis.

Severe aortic regurgitation from aortic valve perforation in Libman-Sacks endocarditis: Incremental value of 3D TEE.

A 43-year-old woman with stroke and systemic lupus erythematosus was found to have severe eccentric aortic regurgitation. Three-dimensional transesophageal echocardiogram further defined the origin of the regurgitant jet through a perforation in the left coronary cusp next to a mass with a tiny stalk attached to the left coronary cusp.

[Superinfection of Libman-Sacks endocarditis in a lupus patient: a case report]. / Surinfection d'une endocardite de Libman-Sacks chez une patiente lupique: rapport de cas.

Libman-Sacks endocarditis is an uncommon cardiac manifestation of lupus, characterized by noninfective heart valve vegetations. Most patients are asymptomatic. However, clinical manifestations of acute forms can mimic infectious endocarditis and complicate both differential diagnosis and treatment. We here report the case of a 28-year-old female patient with lupus erythematosus followed up from 2018. She had signs and symptoms supporting the diagnosis of infective endocarditis. Assessments allowed for the diagnosis of superinfection Libman-Sacks endocarditis. The patient died despite the combination of bi-antibiotic therapy and corticosteroids.

Non-bacterial thrombotic endocarditis: A state-of-the-art contemporary review.

Non-bacterial thrombotic endocarditis (NBTE), also known as Libman-Sacks, marantic, thrombotic, or verrucous endocarditis, is a form of non-infective endocarditis that affects cardiac structures in patients who have predisposing underlying conditions.1 As it is rarely encountered in routine clinical practice, the condition may be overlooked or misdiagnosed. On the other hand, other similar clinical entities might be erroneously labeled as NBTE. Notwithstanding its ostensibly uncommon appearance in clinical practice, our understanding of NBTE has been expanding, especially with the advent of modern and advanced diagnostic tools that facilitate the evaluation process. Herein, we provide a comprehensive review of NBTE, with a focus on the contemporary diagnostic evaluation and management.

A Rare Case of Tricuspid Valve Libman-Sacks Endocarditis in a Pregnant Woman with Primary Antiphospholipid Syndrome.

Antiphospholipid Antibody Syndrome (APS) is a systemic autoimmune disease characterized by acquired hypercoagulability with the possible development of venous, arterial, and microvascular thrombosis. We report a rare case of Libman-Sacks tricuspid valve endocarditis in a 38-year-old pregnant woman at 15 weeks gestation with unknown primary antiphospholipid syndrome. During a routine cardiac examination and echocardiography performed for a previous episode of pleuropericarditis, a large, mobile mass with irregular edges was found at the level of the tricuspid valve. Three main differential diagnoses for intramyocardial mass were examined: tumor, infective endocarditis, and nonbacterial thrombotic endocarditis (NTBE). Cardiac magnetic resonance imaging (CMR) with contrast raised the suspicion of a thrombus. The woman was hospitalized urgently at the Cardiac Intensive Care Unit of the Federico II University Hospital, and anticoagulant and antiplatelet therapy were started. The thrombophilic screening performed and medical history confirmed the diagnosis of primary antibody syndrome (APS). A multidisciplinary consultation with obstetricians, cardiologists, anesthetists, and cardiac surgeons was required. The patient decided not to terminate the pregnancy despite the risk to her health and to undergo cardiac surgery during pregnancy. Histological examination confirmed the presence of nonbacterial thrombotic endocarditis. Weekly obstetric scans were performed after surgery to verify fetal well-being. An emergency cesarean section was performed at the 35th week of gestation due to repeated deceleration and abnormal short-term variability on c-CTG in a pregnancy complicated by fetal growth restriction and gestational hypertension. A newborn weighing 1290 g was born. She was hospitalized in Neonatal Intensive Care and discharged after two months; currently, she enjoys good health. The management of patients with antiphospholipid antibody syndrome has not yet been standardized, but there is a general consensus that patients who do not have thrombocytopenia, thromboembolic phenomena, or pregnancy should not undergo any treatment or should take only low doses of acetylsalicylic acid. In the presence of any of the above conditions, various treatment regimens have been used based on the severity and individuality of the case.

Case report: Mitral valve replacement for Libman-Sacks endocarditis and cerebral embolism of primary antiphospholipid syndrome.

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent arteriovenous thrombosis and/or morbid pregnancy. Valve involvement is the most common cardiac manifestation of APS, with lesions characterized by valve thickening and vegetations known as Libman-Sacks endocarditis (LSE). This report discussed a rare case of a 26-year-old young woman diagnosed with primary APS with multiple cerebral infarctions and right middle cerebral artery occlusion that occured 3 years ago. During the investigation, transthoracic echocardiography (TTE) revealed vegetations on both leaflets of the mitral valve with mild to moderate mitral regurgitation. One year following corticosteroid and anticoagulant treatment, mitral valve fibrosis and moderate to severe regurgitation were noted, after which mitral mechanical valve replacement was finally performed. Accordingly, this report suggests that LSE occurrence should be alerted during the examination of APS patients especially in those with cerebrovascular disease. Furthermore, establishing an early diagnosis and conducting close follow-ups are necessary for its timely intervention and treatment.