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Key Clinical Message: Recurrence of valvular involvement may occur after Libman–Sacks endocarditis surgery, emphasizing the need for frequent multivalvular evaluations with echocardiography or more sensitive methods to optimize surgical outcomes. This report presented a 32‐year‐old woman, complaining of recurrent fever and chills. Physical examination revealed the presence of a third heart sound (S3), a pan‐systolic murmur (III/VI) at mitral and tricuspid foci, tachycardia, and fine pulmonary crackles. Transesophageal echocardiography (TEE) revealed severe mitral regurgitation (MR) and moderate tricuspid regurgitation (TR) with vegetations on the mitral valve. Initially, intravenous antibiotic therapy was started simultaneously with diagnostic studies. Despite a positive TEE, negative blood cultures on three separate occasions precluded meeting the diagnostic criteria outlined in the modified Duke criteria. Moreover, the patient's condition continued to deteriorate after antibiotic therapy, leading to the diagnosis of Libman–Sacks endocarditis. The patient was considered a candidate for mitral valve surgery. All vegetations were completely debrided and then the mitral valve was reconstructed. Follow‐up post‐surgery echocardiography revealed the absence of MR and mitral stenosis (MS). Four months later, the patient presented again complaining of fatigue, dyspnea, lower extremity edema, and ascites with evidence of pulmonary hypertension and right heart failure on physical examination. TEE was performed, which revealed severe MR, severe TR, detached artificial chordae, and blood leak from the perforated pericardial patch. Therefore, she was necessitated for valvular surgery and underwent mitral and tricuspid valve surgery. The mitral ring and perforated pericardial patch were removed, and a mitral prosthetic valve was implanted. In addition, the tricuspid valve was repaired. Follow‐up post‐surgery echocardiography revealed the absence of MR and TR. To our knowledge, this is the first case of LSE recurrence with multi‐valvular involvement. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Noninfective endocarditis, particularly in association with systemic autoimmune diseases like systemic lupus erythematosus (SLE), presents a diagnostic challenge due to its nonspecific symptoms and the potential for severe cardiac complications. We describe a case of a 16-year-old female diagnosed with SLE who presented with fever, malaise, and a new heart murmur. Subsequent investigations revealed vegetations on the mitral valve consistent with noninfective endocarditis without any evidence of infectious etiology. This case underscores the importance of considering noninfective endocarditis in the differential diagnosis for patients with systemic autoimmune disorders presenting with fever and cardiac symptoms. Early recognition and management are crucial to prevent serious outcomes.

Objectives 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-β2-glycoprotein 1 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 a specific serology, namely IgG isotype aPL antibodies and triple positivity. Our data suggest that pAPS-HVD represents a high-risk subgroup of APS patients. [ABSTRACT FROM AUTHOR]

Initially described in 1936, non-bacterial thrombotic endocarditis (NBTE) is a rare entity involving sterile vegetations on cardiac valves. These vegetations are usually small and friable, typically associated with hypercoagulable states of malignancy and inflammatory diseases such as systemic lupus erythematosus. Diagnosis remains challenging and is commonly made post-mortem although standard clinical methods such as echocardiography (transthoracic and transesophageal) and magnetic resonance imaging may yield the clinical diagnosis. Prognosis of NBTE is poor with very high morbidity and mortality usually related to the serious underlying conditions and high rates of systemic embolization. Therapeutic anticoagulation with unfractionated heparin has been described as useful for short term prevention of recurrent embolic events in patients with NBTE but there are no guidelines for management of this disease.

A 14-year-old girl, who is a known case of systemic lupus erythematosus and lupus nephritis, was admitted with complaints of fever for 10 days. Clinical examination did not show any focus of infection. Blood culture and sensitivity showed Staphylococcus aureus septicemia. She was started on antibiotics and subjected to routine transthoracic echocardiography. Transthoracic echocardiography showed Libman–Sacks vegetations (also known as nonbacterial thrombotic endocarditis) on all the cusps of both mitral and tricuspid valves, along with sessile freely mobile infective vegetation attached to the right ventricular free wall, and pulmonic valve abscess moving to and fro about the pulmonic annulus. Surgery was deferred due to poor general condition, and the patient was continued on parenteral therapy. The girl succumbed to her illness on the 6th day of her illness.

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.

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.

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. [ABSTRACT FROM AUTHOR]

Patients with Antiphospholipid syndrome (APLS) are at high risk for both bleeding and thrombotic complications during cardiac surgery involving cardiopulmonary bypass (CPB). In this case we present a patient with APLS and Immune Thrombocytopenic Purpura who successfully underwent aortic valve replacement (AVR) with CPB despite recent craniotomy for subdural hematoma evacuation. Anticoagulation for CPB was monitored by targeting an Activated Clotting Time (ACT) that was 2× the upper limit of normal. A multidisciplinary approach was essential in ensuring a safe and successful operation.

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. [ABSTRACT FROM AUTHOR]

Background: Nonbacterial thrombotic endocarditis is characterized by the presence of organized thrombi on cardiac valves, often associated with hypercoagulable states. There is a paucity of data regarding the predictors of mortality in patients with nonbacterial thrombotic endocarditis. Our primary aim was to identify predictors of in-hospital mortality in patients with nonbacterial thrombotic endocarditis. Methods: A systematic literature review of all published cases and case series was performed until May 2018 according to Preferred Reporting Items for Systematic Review and Meta-analyses statement guidelines. We applied random forest machine learning model to identify predictors of in-patient mortality in patients with nonbacterial thrombotic endocarditis. Results: Our search generated a total of 163 patients (mean age, 46 ± 17 years; women, 69%) with newly diagnosed nonbacterial thrombotic endocarditis. The in-hospital mortality rate in the study cohort was 30%. Among the patients who died in the hospital, initial presentation of pulmonary embolism (12.2 vs. 2.6%), splenic (38.7 vs. 10.5%), and renal (40.8 vs. 9.6%) infarcts were higher compared to patients alive at the time of discharge. Higher rates of malignancy (71.4 vs. 39.4%, P = .0003) and lower rates of antiphospholipid syndrome (8.1 vs. 48.2%, P = .0001) were noted in deceased patients. Random forest machine learning analysis showed that older age, presence of antiphospholipid syndrome, splenic infarct, renal infarct, peripheral thromboembolism, pulmonary embolism, myocardial infarction, and mitral valve regurgitation were significantly associated with increased risk of in-hospital mortality. Conclusion: Patients admitted with nonbacterial thrombotic endocarditis have a high rate of in-hospital mortality. Factors including older age, presence of antiphospholipid syndrome, splenic/renal infarct, lower limb thromboembolism, pulmonary embolism, myocardial infarction, and mitral valve regurgitation were significantly associated with increased risk of in-hospital mortality in patients with nonbacterial thrombotic endocarditis. [ABSTRACT FROM AUTHOR]

Nonbacterial thrombotic endocarditis (NBTE) is a form of endocarditis associated with malignancy or autoimmune disorders. Diagnosis remains a challenge as patients are often asymptomatic up to embolic events or rarely, valve dysfunction. We report a case of NBTE with uncommon clinical presentation and identified with multimodal echocardiography. An 82‑year‑old man presented to our outpatient clinic reporting dyspnea. Past medical history included hypertension, diabetes, kidney disease, and unprovoked deep‑vein thrombosis. On physical examination, he was apyretic, mildly hypotensive, and hypoxemic, had a systolic murmur and lower limbs edema. Transthoracic echocardiography revealed severe mitral regurgitation due to verrucous thickening of the free margin of both leaflets, increased pulmonary pressure, and dilated inferior vena cava. Multiple blood cultures were negative. Transesophageal echocardiography confirmed “thrombotic” thickening of mitral leaflets. Nuclear investigations were highly suggestive of multi‑metastatic pulmonary cancer. We did not further proceed with the diagnostic workup and prescribed palliative care. Lesions seen on echocardiography were suggestive of NBTE: they involved both sides of mitral leaflets, close to the edges, had irregular shape and echo density, a broad base, and no independent motion. Criteria for infective endocarditis were not met and the final diagnosis was paraneoplastic NBTE due to underlying lung cancer. We remark the lack of definitive recommendations about the treatment of NBTE and the only role of anticoagulation to prevent systemic embolism. We have reported a case of NBTE presenting with atypical symptoms and likely related to the prothrombotic state induced by underlying lung cancer. Provided the unconclusive microbiological tests, multimodal imaging has played a crucial role in the final diagnosis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract Background Adult-onset Still’s disease (AOSD) should be considered in the differential diagnosis of patients with endocarditis, with or without a cardiac decompensation. Case presentation We report the case of a 68-year-old Caucasian male diagnosed with AOSD after an initial acute manifestation of endocarditis with severe aortic acute manifestation of endocarditis with severe aortic insufficiency. The histological findings revealed Libman–Sacks endocarditis. He was treated with the IL-1 receptor inhibitor anakinra. Two years later the patient developed a symptomatic dilated cardiomyopathy with reduced ejection fraction (23.5%) and functional anti-beta-1-adrenergic receptor antibodies, which was initially treated with plasmapheresis; anakinra was maintained. While his AOSD symptoms responded well, our patient presented with recurrent arthritis in multiple joints, dual-energy CT showed urate deposition compatible with a gouty arthropathy. Over 7 years, he presented with recurrent episodes of arthritis and the adjustment of dosages of colchicine and febuxostat was needed. In 2018, our patient died due to a deterioration of his underlying cardiac disease. Conclusions Only two cases with initial endocarditis prior to AOSD diagnosis have been published, and we are not aware of any other cases reporting -β1AR-Ab development with DCM and gout in the setting of AOSD treated with anakinra.

We aimed to describe a case of neovascular glaucoma (NVG) as a first presenting sign of catastrophic antiphospholipid syndrome (CAPS) with heart valve aseptic vegetations known as Libman-Sacks endocarditis. A 39-year-old man was referred for left eye decreased visual acuity and pain, upon examination left eye high intraocular pressure; rubeosis iridis of both eyes (BE); and prominent retinal ischemia. Clinical and fluorescein angiography findings established the diagnosis of left eye NVG with vaso-occlusive disease in BE. Magnetic resonance imaging of the head showed widespread ischemic lesions and hemorrhagic foci. The transesophageal echocardiogram showed 2 big mitral valve lesions consistent with the diagnosis of Libman-Sacks endocarditis. Laboratory and clinical diagnosis of CAPS and suspected SLE was confirmed, and treatment with anticoagulants and IV steroids was initiated. This case demonstrates that severe vaso-occlusive retinopathy with severe brain ischemia should raise the suspicion of systemic autoimmune pro-coagulative diseases with heart valve aseptic vegetations.

Libman-Sacks endocarditis is a rare cardiac manifestation of anti-phospholipid syndromes, in which non-infectious thrombotic vegetations are found on the heart valves. Most patients are asymptomatic whereas the risk of thromboembolism is considerable. Diagnostic work-up is based on questioning and clinical examination data looking for extracardiac signs, biological data and also on imaging, and, above all, echocardiography. We report the case of a 47-year-old female patient with no known history who is admitted to hospital with paresthesia of the right hemi-body associated with dysarthria. Cerebral CT scan confirms a paraventricular ischemic stroke. The etiological work-up for stroke is negative except the transesophageal echocardiogram which reveals mitral valve vegetations. Further investigations lead to the diagnosis of Libman-Sacks endocarditis. Treatment with Coumadin is started, with a target INR of between 2 and 3, as recommended. The clinical course was favourable, with stable lesions on transoesophageal echocardiography carried out later. [ABSTRACT FROM AUTHOR]

Abstract Background Antiphospholipid syndrome is an antibody mediated pro-thrombotic state leading to various arterial and venous thromboses. The syndrome can be either primary or secondary to other autoimmune diseases, commonly systemic lupus erythematosus. Cardiac involvement, in particular valvular disease is common in patients with antiphospholipid syndrome, occurring in about a third of these patients. Valvular diseases associated with antiphospholipid syndrome often occur as valve thickening and non-bacterial vegetation or Libman-Sacks endocarditis. Deposits of antiphospholipid immunoglobulin and complement components are commonly observed in the affected valves, suggesting an inflammatory process resulting in valvular vegetation and thickening. Libman-Sacks endocarditis has a high propensity towards mitral valve, although haemodynamically significant valvular dysfunction is rare. Case presentation We present a successful aortic valve replacement with cardiopulmonary bypass in a 48 years old lady with antiphospholipid syndrome, who has severe aortic regurgitation as a result of Libman-sacks endocarditis. Antiphospholipid antibodies were positive and the clinical data showed both negative cultures and infective parameters. Surgically resected vegetations revealed sterile fibrinous and verrucous vegetations on aortic valve. Valve replacement and the course of cardiopulmonary bypass was uneventful, and the patient was discharged well. Conclusions Classically Libman-Sacks endocarditis is often and more commonly associated with autoimmune diseases such as systemic lupus erythematosus, although it can occur in both primary and secondary antiphospholipid syndrome. It is not a common entity, and it is a frequent underestimated disease as most clinicians do not routinely screen for valvular lesion in patients with antiphospholipid syndrome unless they are symptomatic. However, due to its high prevalence of cardiac involvement, clinicians should have a high index of suspicion in the attempt to minimize cardiovascular and haemodynamic complications. Valve surgery in patients with antiphospholipid syndrome carries considerable early and late morbidity and mortality, usually caused by thromboembolic and bleeding events. The perioperative anticoagulation management and haemostatic aspect of antiphospholipid syndrome present an exceptional challenges to clinicians, surgeons, anaesthetists and laboratory personnel.

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.

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. [ABSTRACT FROM AUTHOR]

Patients with Antiphospholipid syndrome (APLS) are at high risk for both bleeding and thrombotic complications during cardiac surgery involving cardiopulmonary bypass (CPB). In this case we present a patient with APLS and Immune Thrombocytopenic Purpura who successfully underwent aortic valve replacement (AVR) with CPB despite recent craniotomy for subdural hematoma evacuation. Anticoagulation for CPB was monitored by targeting an Activated Clotting Time (ACT) that was 2× the upper limit of normal. A multidisciplinary approach was essential in ensuring a safe and successful operation. [ABSTRACT FROM AUTHOR]

Aortic insufficiency is commonly observed in rheumatologic diseases such as ankylosing spondylitis, systemic lupus erythematosus, antiphospholipid syndrome, Behçet's disease, granulomatosis with polyangiitis, and Takayasu arteritis. Aortic insufficiency with an underlying rheumatologic disease may be caused by a primary valve pathology (leaflet destruction, prolapse or restriction), annular dilatation due to associated aortitis or a combination of both. Early recognition of characteristic valve and aorta morphology on cardiac imaging has both diagnostic and prognostic importance. Currently, echocardiography remains the primary diagnostic tool for aortic insufficiency. Complementary use of computed tomography, cardiac magnetic resonance imaging and positron emission tomography in these systemic conditions may augment the assessment of underlying mechanism, disease severity and identification of relevant non-valvular/extracardiac pathology. We aim to review common rheumatologic diseases associated with aortic insufficiency and describe their imaging findings that have been reported in the literature.

Nonbacterial thrombotic endocarditis (NBTE) is a rare but serious complication, particularly in patients with malignancies like acute myeloid leukemia (AML), where a hypercoagulable state increases the risk of embolic events. This case report describes a rare and complex presentation of marantic endocarditis in a 78-year-old female with relapsed AML. The uniqueness of this case lies in the intersection of a hypercoagulable state induced by AML and the resultant NBTE, leading to recurrent embolic strokes, despite oral anticoagulation. This case contributes to the scientific literature by highlighting the diagnostic and therapeutic challenges in managing NBTE in patients with hematologic malignancies. The patient had multiple ED visits, initially with concerns of persistent back spasms, and was diagnosed with venous thromboembolism and started on apixaban.​ She then returned to our ED with visual disturbances and headaches. Significant clinical findings included right hemianopsia and circulating blasts on laboratory tests, indicative of AML relapse. Imaging studies revealed multiple small acute cerebral infarctions and evidence of thrombus affecting the mitral and aortic valves. The patient was diagnosed with NBTE and treated with systemic anticoagulation using warfarin, bridged with enoxaparin sodium. She remained stable during hospitalization and was discharged with follow-up care coordinated among her oncology, neurology, and cardiology teams. NBTE is an important consideration in a differential diagnosis in patients with cancer causing a hypercoagulable state such as AML, particularly in patients who present with neurological symptoms. High index of suspicion and a multidisciplinary approach are essential for the timely diagnosis and management of NBTE to prevent further thromboembolic complications., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Rankin et al.)

BackgroundLibman-Sacks endocarditis, characterized by Libman-Sacks vegetations, is common in patients with systemic lupus erythematosus and is commonly complicated with embolic cerebrovascular disease. Thus, accurate detection of Libman-Sacks vegetations may lead to early therapy and prevention of their associated complications. Although two-dimensional (2D) transesophageal echocardiography (TEE) has high diagnostic value for detection of Libman-Sacks vegetations, three-dimensional (3D) TEE may allow improved detection, characterization, and clinical correlations of Libman-Sacks vegetations.MethodsTwenty-nine patients with systemic lupus erythematosus (27 women; mean age, 34 ± 12 years) prospectively underwent 40 paired 3D and 2D transesophageal echocardiographic studies and assessment of cerebrovascular disease manifested as acute clinical neurologic syndromes, neurocognitive dysfunction, or focal brain injury on magnetic resonance imaging. Initial and repeat studies in patients were intermixed in a blinded manner with paired studies from healthy controls, deidentified, coded, and independently interpreted by experienced observers unaware of patients' clinical and imaging data.ResultsThe results of 3D TEE compared with 2D TEE were more often positive for mitral or aortic valve vegetations, and 3D TEE detected more vegetations per study and determined larger sizes of vegetations (P ≤ .03 for all). Also, 3D TEE detected more vegetations on the anterior mitral leaflet, anterolateral and posteromedial scallops, and ventricular side or both atrial and ventricular sides of the leaflets (P

Libman-Sacks endocarditis is usually associated with systemic lupus erythematosus and malignancies. It usually presents between ages 40 and 70 years. Our case was a seven year old child who was initially suspected and treated on lines of rat bite fever endocarditis based on history of rat bite but on further investigations patient was diagnosed with libman sacks endocarditis. High index of suspicion should be there for Libman-Sacks endocarditis for early diagnosis and treatment in cases with positive family history.

Background  Systemic lupus erythematosus (SLE) valvulopathy can manifest as a spectrum of pathologies and treatment of severe valvular dysfunction thus far has been surgical. However, surgery in patients with SLE is frequently associated with high morbidity and mortality due to the presence of significant co-morbidities. Case summary  We report the case of a 41-year-old woman with SLE and anti-phospholipid syndrome with extensive co-morbidities including lupus nephritis, pancytopaenia, cerebrovascular accident, and severe airway obstruction from ipsilateral lung collapse and bronchiectasis. She had severe mitral regurgitation (MR) from Libman–Sacks endocarditis and in recent months developed heart failure with progressive exertional dyspnoea from New York Heart Association (NYHA) functional Class from New York Heart Association (NYHA) functional class II to III. In addition, there was progressive left ventricular dilatation and reduction in left ventricular ejection fraction. In view of the high surgical risk, she underwent transcatheter edge-to-edge repair (TEER) of the mitral valve with the MitraClip system. At 1-month follow-up, she was back to NYHA functional Class II with mild MR. Discussion Our case demonstrates that in select patient with suitable anatomy, TEER is a potential treatment option for severe MR from SLE valvulopathy. [ABSTRACT FROM AUTHOR]

Background: Adult-onset Still’s disease (AOSD) should be considered in the differential diagnosis of patients with endocarditis, with or without a cardiac decompensation. Case presentation: We report the case of a 68-year-old Caucasian male diagnosed with AOSD after an initial acute manifestation of endocarditis with severe aortic acute manifestation of endocarditis with severe aortic insufficiency. The histological findings revealed Libman–Sacks endocarditis. He was treated with the IL-1 receptor inhibitor anakinra. Two years later the patient developed a symptomatic dilated cardiomyopathy with reduced ejection fraction (23.5%) and functional anti-beta-1-adrenergic receptor antibodies, which was initially treated with plasmapheresis; anakinra was maintained. While his AOSD symptoms responded well, our patient presented with recurrent arthritis in multiple joints, dual-energy CT showed urate deposition compatible with a gouty arthropathy. Over 7 years, he presented with recurrent episodes of arthritis and the adjustment of dosages of colchicine and febuxostat was needed. In 2018, our patient died due to a deterioration of his underlying cardiac disease. Conclusions: Only two cases with initial endocarditis prior to AOSD diagnosis have been published, and we are not aware of any other cases reporting -β1AR-Ab development with DCM and gout in the setting of AOSD treated with anakinra. [ABSTRACT FROM AUTHOR]

Antiphospholipid syndrome (APS) is a clinical disorder that creates an increased risk of arterial or venous thrombotic events or pregnancy-associated complications and includes the presence of autoantibodies against negatively charged phospholipids. This syndrome is often associated with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE). Libman-Sacks endocarditis is a form of non-bacterial thrombotic endocarditis and is infrequently seen in APS. There are few data documenting the echocardiographic response of APS valve disease to medical treatment. This is an unusual case of a young female patient with SLE and APS who had chorea and non-bacterial thrombotic aortic valve endocarditis. Echocardiography revealed that the vegetation had receded after a combination of warfarin and immunosuppressive therapy.

Background: Libman-Sacks endocarditis (LSE) is characterized by sterile lesions that commonly affect the aortic and mitral heart valves. Antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) have been associated with LSE. Cardiac manifestations including LSE could be interrelated with other manifestations and early diagnosis could help in preventing further complications. Case presentation: Here, we report three cases of LSE in SLE patients with secondary APS. All patients presented with neurological manifestations and LSE was diagnosed by Transesophageal echocardiography (TEE). All three patients were treated for the underlying disease and also received anticoagulant therapy. Conclusion: In all patients with SLE and secondary APS, LSE should be considered if a patient manifests any evidence of neurologic involvement.

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We aimed to describe a case of neovascular glaucoma (NVG) as a first presenting sign of catastrophic antiphospholipid syndrome (CAPS) with heart valve aseptic vegetations known as Libman-Sacks endocarditis. A 39-year-old man was referred for left eye decreased visual acuity and pain, upon examination left eye high intraocular pressure; rubeosis iridis of both eyes (BE); and prominent retinal ischemia. Clinical and fluorescein angiography findings established the diagnosis of left eye NVG with vaso-occlusive disease in BE. Magnetic resonance imaging of the head showed widespread ischemic lesions and hemorrhagic foci. The transesophageal echocardiogram showed 2 big mitral valve lesions consistent with the diagnosis of Libman-Sacks endocarditis. Laboratory and clinical diagnosis of CAPS and suspected SLE was confirmed, and treatment with anticoagulants and IV steroids was initiated. This case demonstrates that severe vaso-occlusive retinopathy with severe brain ischemia should raise the suspicion of systemic autoimmune pro-coagulative diseases with heart valve aseptic vegetations. [ABSTRACT FROM AUTHOR]

Background: Antiphospholipid syndrome is an antibody mediated pro-thrombotic state leading to various arterial and venous thromboses. The syndrome can be either primary or secondary to other autoimmune diseases, commonly systemic lupus erythematosus. Cardiac involvement, in particular valvular disease is common in patients with antiphospholipid syndrome, occurring in about a third of these patients. Valvular diseases associated with antiphospholipid syndrome often occur as valve thickening and non-bacterial vegetation or Libman-Sacks endocarditis. Deposits of antiphospholipid immunoglobulin and complement components are commonly observed in the affected valves, suggesting an inflammatory process resulting in valvular vegetation and thickening. Libman-Sacks endocarditis has a high propensity towards mitral valve, although haemodynamically significant valvular dysfunction is rare.Case Presentation: We present a successful aortic valve replacement with cardiopulmonary bypass in a 48 years old lady with antiphospholipid syndrome, who has severe aortic regurgitation as a result of Libman-sacks endocarditis. Antiphospholipid antibodies were positive and the clinical data showed both negative cultures and infective parameters. Surgically resected vegetations revealed sterile fibrinous and verrucous vegetations on aortic valve. Valve replacement and the course of cardiopulmonary bypass was uneventful, and the patient was discharged well.Conclusions: Classically Libman-Sacks endocarditis is often and more commonly associated with autoimmune diseases such as systemic lupus erythematosus, although it can occur in both primary and secondary antiphospholipid syndrome. It is not a common entity, and it is a frequent underestimated disease as most clinicians do not routinely screen for valvular lesion in patients with antiphospholipid syndrome unless they are symptomatic. However, due to its high prevalence of cardiac involvement, clinicians should have a high index of suspicion in the attempt to minimize cardiovascular and haemodynamic complications. Valve surgery in patients with antiphospholipid syndrome carries considerable early and late morbidity and mortality, usually caused by thromboembolic and bleeding events. The perioperative anticoagulation management and haemostatic aspect of antiphospholipid syndrome present an exceptional challenges to clinicians, surgeons, anaesthetists and laboratory personnel. [ABSTRACT FROM AUTHOR]

L'endocardite de Libman-Sacks est une complication rare du syndrome des antiphospholipides. L'anticoagulation par anti-vitamine K est le traitement de référence, parfois associée à une chirurgie. Dans les dernières recommandations EULAR, l'utilisation des anticoagulants oraux directs est déconseillée pour la prise en charge du syndrome des antiphospholipides, en particulier en cas de profil à haut risque. Nous rapportons le cas d'une patiente de 63 ans atteinte de sclérose en plaque et d'un syndrome des antiphospholipides ayant présenté une endocardite mitrale de Libman-Sacks compliquée d'accidents vasculaires ischémiques après deux années d'apixaban. La réintroduction de tinzaparine puis de warfarine au long cours et deux mois d'hydroxychloroquine ont permis une amélioration des lésions valvulaires. À notre connaissance, il s'agit du premier cas d'endocardite de Libman-Sacks survenue sous apixaban pour le traitement d'un syndrome des antiphospholipides. Cette atteinte grave conforte l'idée que les anticoagulants oraux directs ne sont pas à utiliser dans la prévention des évènements thrombotiques au cours du syndrome des antiphospholipides. Libman-Sacks endocarditis is a rare complication of antiphospholipid syndrome. Anti-vitamin K therapy is the standard treatment, although valvular replacement surgery may be required in some severe cases. In the latest EULAR recommendations, it is advised not to use direct oral anticoagulants in the management of antiphospholipid syndrome, especially of high-risk profile. We present a case of a mitral Libman-Sacks endocarditis complicated with multiple strokes occurring in the setting of an antiphospholipid syndrome with triple positive antibody profile in a 63-year-old woman with multiple sclerosis. She was previously treated with apixaban for two years. Tinzaparin followed by prolonged warfarine treatment and two months of hydroxychloroquine resulted in valvular improvement. To our knowledge, this is the first case of Libman-Sacks endocarditis occurring during apixaban therapy in a patient with antiphospholipid syndrome. This severe case highlights the inefficiency of direct oral anticoagulants to prevent thrombotic events in the antiphospholipid syndrome. [ABSTRACT FROM AUTHOR]

Libman-Sacks endocarditis (LSE) is a rare complication of systemic lupus erythematosus (SLE), characterized by noninfectious vegetation on normal heart valves. We present the case of a 20-year-old woman with SLE and a congenital valve malformation. Despite a year of effective SLE treatment, she later developed stage IV dyspnea, chest pain, and signs of right heart failure. Investigations revealed active lupus, mitral valve vegetation, agenesis of the posterior mitral leaflet, and severe mitral insufficiency. The patient was treated with corticosteroids, antibiotics, anticoagulants, and symptomatic heart failure management. Despite initial improvement, her condition deteriorated, and she did not respond to resuscitation. While LSE often responds well to treatment, severe valvulopathy, particularly with congenital valve defects, can result in fatal outcomes., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: Our manuscript is in preprint status after being submitted to a journal and rejected., (Copyright © 2024, Zaher et al.)

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.

Initially described in 1936, non-bacterial thrombotic endocarditis (NBTE) is a rare entity involving sterile vegetations on cardiac valves. These vegetations are usually small and friable, typically associated with hypercoagulable states of malignancy and inflammatory diseases such as systemic lupus erythematosus. Diagnosis remains challenging and is commonly made post-mortem although standard clinical methods such as echocardiography (transthoracic and transesophageal) and magnetic resonance imaging may yield the clinical diagnosis. Prognosis of NBTE is poor with very high morbidity and mortality usually related to the serious underlying conditions and high rates of systemic embolization. Therapeutic anticoagulation with unfractionated heparin has been described as useful for short term prevention of recurrent embolic events in patients with NBTE but there are no guidelines for management of this disease.

Systemic lupus erythematosus (SLE) is a potentially fatal autoimmune disease that remains frequently underdiagnosed due to the heterogeneity of its symptoms. Lack of specific features at the initial presentation often results in delayed diagnosis. We present the case of a 58 years old SLE patient with renal, neurological, hematological and articular involvement and associated antiphospholipid syndrome, whose first manifestation of the disease occurred 20 years before the diagnosis could be made. SLE is a disease with a wide array of symptoms. As such, unless a strong index of suspicion is maintained, the disease diagnosis and specific treatment may be easily delayed.

Although neutrophil extracellular traps (NETs) have been highlighted in several systemic inflammatory diseases, their clinical correlates and potential pathological role remain obscure. Herein, we describe a dramatic onset of systemic lupus erythematosus (SLE) with clear-cut pathogenic implications for neutrophils and NET formation in a young woman with cardiac (Libman–Sacks endocarditis) and central nervous system (psychosis and seizures) involvement. Despite extensive search, circulating antiphospholipid autoantibodies, a hallmark of Libman–Sacks endocarditis, could not be detected. Instead, we observed active NET formation in the tissue of the mitral valve, as well as in the circulation. Levels of NET remnants were significantly higher in serially obtained sera from the patient compared with sex-matched blood donors (p = .0011), and showed a non-significant but substantial correlation with blood neutrophil counts (r = 0.65, p = .16). The specific neutrophil elastase activity measured in serum seemed to be modulated by the provided immunosuppressive treatment. In addition, we found anti-Ro60/SSA antibodies in the cerebrospinal fluid of the patient but not NET remnants or increased elastase activity. This case illustrates that different disease mechanisms mediated via autoantibodies can occur simultaneously in SLE. NET formation with release of cytotoxic NET remnants is a candidate player in the pathogenesis of this non-canonical form of Libman–Sacks endocarditis occurring in the absence of traditional antiphospholipid autoantibodies. The case description includes longitudinal results with clinical follow-up data and a discussion of the potential roles of NETs in SLE.

Aortic valve (AV) thrombus, a rare complication of antiphospholipid syndrome (APLS), is important to distinguish from Libman-Sacks endocarditis because of its responsiveness to anticoagulation. This may be attributed to immunopathologic differences underpinning their development. We present the case of a 45-year-old woman with high-risk primary APLS who developed an AV mass and was taken for valvular repair surgery but found to have pure thrombus and normal valve leaflets. In such cases, a trial of conservative management with anticoagulation may be adequate. Echocardiography, computed tomography and MRI findings suggestive of thrombus without endocarditis are presented. A literature review of histopathologic, imaging and treatment implications of pure AV thrombus in the context of APLS is included. [ABSTRACT FROM AUTHOR]

Objective: Treatment of Libman–Sacks (LS) endocarditis in patients with systemic lupus erythematosus (SLE) is challenging due to the lack of data. This study aimed to identify the clinical characteristics of SLE patients and LS endocarditis, and to investigate the treatment and prognosis of LS endocarditis. Methods: Of all the patients with SLE who underwent echocardiography between 2010 and 2019, 11 and 29 patients with and without LS endocarditis, respectively, were included. We compared the inflammatory and thrombotic profiles between patients with and without LS endocarditis, and investigated the treatment and long-term outcome of LS endocarditis. Results: No significant differences were observed in disease activity, clinical characteristics and inflammatory marker levels between patients with and without LS endocarditis. Patients with LS endocarditis had a significantly higher prevalence of antiphospholipid antibody (aPL) but a lower prevalence of SLE-specific antibody. Triple positivity of aPL was found in 72.7% and 13.8% of patients with and without LS endocarditis, respectively. Of 11 patients with LS endocarditis, six patients received anticoagulation therapy, and five patients received augmented immunosuppressive therapies. One patient who did not receive anticoagulation therapy developed cerebral infarction. Nine (82%) patients with LS endocarditis were classified as having antiphospholipid syndrome (APS). Despite the residual vegetation and valve dysfunction, surgical intervention was not required during the follow-up period of 56.8 months. Conclusion: A significant correlation was observed between APS and LS endocarditis. Anticoagulation therapy should be considered to prevent thromboembolic complications in SLE patients with LS endocarditis. [ABSTRACT FROM AUTHOR]

Hypertrophic obstructive cardiomyopathy in Libman–Sacks endocarditis is quite rare and the correct etiological relationship between them is unknown. Some changes may cause a secondary disorganization of the ordinary muscle structure, making a disarray pattern with irregular interwoven myocyte fibers. This case report describes one of the first cases of ventricular septal myectomy and mitral valve replacement for hypertrophic obstructive cardiomyopathy and mitral valve regurgitation associated with Libman–Sacks endocarditis. [ABSTRACT FROM AUTHOR]

Endocarditis is most frequently infective in origin, and thus, when a patient presents with a clinical picture suggestive of endocarditis, an extensive work up aimed at finding the infectious agent is warranted. Among systemic lupus erythematosus (SLE) patients, cardiovascular disease is prevalent in more than 50% of patients including valvular disease and non-infective endocarditis, known as Libman–Sacks (LS) endocarditis. The prevalence of LS syndrome among SLE patients with secondary antiphospholipid syndrome (APS) is higher than in SLE without APS. Here, we present a case of a patient diagnosed with primary APS who presented with hemoptysis and a cardiac murmur. The diagnosis of SLE was established following the findings of non-infective verrucous vegetations together with diffuse alveolar hemorrhage (DAH). Treatment with high-dose corticosteroids and intravenous immunoglobulins yielded substantial resolution of the vegetations and regression of the DAH. Hence, aortic valve replacement was successfully performed as an elective procedure and without any postoperative complications. The patient is in remission after a 6-month follow-up. The clinical findings of DAH and double valve non-infectious endocarditis prompted the diagnosis of SLE with secondary APS. [ABSTRACT FROM AUTHOR]

Libman-Sacks endocarditis is a nonbacterial thrombotic endocarditis, characterized by the deposition of sterile platelet thrombi on heart valves, aortic and mitral. It is associated with systemic lupus erythematosus and the clinical manifestation is typically asymptomatic. It is associated with disease duration, lupus activity and antiphospholipid antibodies. The diagnosis is by echocardiography and the definitive diagnosis can be made pathologically by the demonstration of vegetations on autopsy or surgical specimens. This paper reports the case of a 62-year old patient with asthenia, adynamia and dyspnea of medium efforts; the echocardiography showed vegetations and patient was underwent to surgical treatment with resection of vegetations, diagnosis was confirmed by pathology. [ABSTRACT FROM AUTHOR]

Libman-Sacks endocarditis is a rare cardiac manifestation systemic lupus erythematosus, in which there is a sterile vegetation in the heart valves. There is a significant risk of infective endocarditis. Our patient was a 38 year old woman with persistent fever from two months with inflammatory polyarthralgia, fixed at the wrists and ankles. She was febrile at 39 °C, had a mitral systolic murmur 2/6 and painful swell ing of the wrists and ankles. We have objectified an inflammatory syndrome, blood cultures were negative. The dosage of anti-nuclear antibody was positive with a mottled appearance, as well as anti-DNA antibodies. The Doppler echocardiography had objectified vegetations in the mitral and aortic valves. Clinical, biological and morphological improvements were obtained after antibiotic and corticosteroid combination. We can conclude that Libman-Sacks endocarditis evolution is favorable in the absence of an associated antiphospholipid syndrome (APS). Always fear in all cases a surinfection. The treatment is based on the combination antibiotic-corticosteroid-synthetic antimalarial. [ABSTRACT FROM AUTHOR]