Nonbacterial Thrombotic Endocarditis Research Articles

Clinical, echocardiographic, and pathologic characteristics of patients with pathology-proven nonbacterial thrombotic endocarditis

Abstract Background Non-bacterial thrombotic endocarditis (NBTE) is a non-infectious condition characterized by thrombotic and/or inflammatory depositions involving the cardiac valves. It is a challenging diagnosis of exclusion. Purpose This study aimed to better characterize patients with pathologically proven NBTE and assess its clinical, echocardiographic characteristics and prognostic implications. Methods We retrospectively identified patients with pathology-proven NBTE at a single center. Patients with positive preoperative blood cultures, a history of active or treated infective endocarditis, or identification of valvular organisms by special stains were excluded from the study. Patients with pathology reports of valve perforation or chordal rupture were also excluded because of the high likelihood of an infectious etiology for these lesions. Results A total of 53 patients with NBTE (median age 57 years; 61% females) were identified. In this cohort, 22% had a history of malignancy, 30% had connective tissue disease, 32% had coronary artery disease, and 11% prior history of NBTE. Neurological events accounted for 18 (34%) of the presenting symptoms (TIA n=17, CVA n=9), while new valvular dysfunction in 7 (13%) and fever in 8 (15%) of patients. Five patients (9%) had NBTE involving more than one valve. NBTE involved the mitral valve in 64%, aortic valve in 38% prosthetic/mechanical valve in 11%, and tricuspid valve in 5%. NBTE was identified at the time of autopsy in 34% of patients. NBTE recurred in 3 patients during follow-up. Excluding patients diagnosed at the time of autopsy, 9% of patients died within 1 year of NBTE diagnosis. Conclusion NBTE was diagnosed pathologically in the presence of hyper-inflammatory states due to critical illness or immunological diseases. It was predominantly on the left side of the heart and was associated with embolic phenomena, either clinically or on imaging. Patients diagnosed with antemortem with NBTE have a high mortality in the first year (9%).

Interplay of Cardiometabolic Syndrome and Biliary Tract Cancer: A Comprehensive Analysis with Gender-Specific Insights.

BTC overall incidence is globally increasing. CCA, including its subtypes, is a form of BTC. MetS, obesity, MASLD, and diabetes are all linked to CCA in interconnected ways. The link between obesity and CCA is less well-defined in Eastern countries as compared to Western. Although more research is needed to determine the relationship between MASLD and extrahepatic CCA (eCCA), MASLD may be a concurrent risk factor for intrahepatic CCA, particularly in populations with established or unidentified underlying liver disease. Interestingly, the risk of biliary tract cancer (BTC) seemed to be higher in patients with shorter diabetes durations who were not treated with insulin. Therefore, early detection and prevention of chronic liver disease, as well as additional intervention studies, will undoubtedly be required to determine whether improvements to MetS, weight loss, and diabetes therapy can reduce the risk and progression of BTC. However, further studies are needed to understand how reproductive hormones are involved in causing BTC and to develop consistent treatment for patients. Finally, it is critical to carefully assess the cardiological risk in BTC patients due to their increased intrinsic cardiovascular risk, putting them at risk for thrombotic complications, cardiovascular death, cardiac metastasis, and nonbacterial thrombotic endocarditis. This review aimed to provide an updated summary of the relation between the abovementioned cardio-metabolic conditions and BTC.

Overview and Future Direction of Embolic Stroke of Undetermined Source from the Insights of CHALLENGE ESUS/CS Registry.

Cryptogenic stroke (CS) accounts for approximately one-fourth of acute ischemic strokes, with most cases derived from embolic etiologies. In 2014, embolic stroke of undetermined source (ESUS) was advocated and the efficacy of anticoagulant therapy was anticipated. However, 3 large-scale clinical trials failed to demonstrate the superiority of direct oral anticoagulants (DOACs) over aspirin, potentially due to the heterogeneous and diverse pathologies of ESUS, including paroxysmal atrial fibrillation (AF), arteriogenic sources such as nonstenotic carotid plaque and aortic complicated lesion (ACL), patent foramen oval (PFO), and nonbacterial thrombotic endocarditis (NBTE) related to active cancer.Transesophageal echocardiography (TEE) is one of the most effective imaging modalities for assessing embolic sources in ESUS and CS. The Mechanisms of Embolic Stroke Clarified by Transesophageal Echocardiography for Embolic Stroke of Undetermined Source/Cryptogenic Stroke (CHALLENGE ESUS/CS) registry is a multicenter registry that enrolled consecutive patients with CS who underwent TEE at 8 hospitals in Japan between April 2014 and December 2016. Their mean age was 68.7±12.8 years, and 455 patients (67.2%) were male. The median National Institutes of Health Stroke Scale (NIHSS) score was 2. Since 7 analyses have been conducted from each institution to date, novel and significant insights regarding embolic origins and pathophysiologies of ESUS and CS were elucidated from this multicenter registry. This review discusses the diagnosis and treatment of ESUS and CS, tracing their past and future directions. Meaningful insights from the CHALLENGE ESUS/CS registry are also referenced and analyzed.

Outcomes and Predictors of Inpatient Mortality for Marantic Endocarditis Complicating Systemic Lupus Erythematosus: Contemporary Nationwide Study From the United States.

Systemic lupus erythematosus (SLE) patients are susceptible to marantic endocarditis (ME) due to a hypercoagulable state. The literature regarding the epidemiology and outcomes of ME in SLE patients is limited. All patients ≥18 years who had SLE with and without ME between 2007 and 2019 were identified from the National Inpatient Sample in the United States (US). Predictors of inpatient mortality for SLE patients with ME were analyzed. Between 2007 and 2019, there were 508,818 hospitalizations for SLE, of which 785 (0.2%) had ME. Of SLE patients with ME, 33 (4.2%) died while hospitalized over the study period. On multivariate analysis, female sex (adjusted odds ratio (aOR), 95% confidence intervals: 24.72 (3.21, 190.27)), age <34 years (aOR: 6.81 (1.80, 25.79)), anemia (aOR: 3.41 (1.12, 10.40)), antiphospholipid syndrome (aOR: 13.50 (3.83, 47.64)), stroke complicating ME (aOR: 9.64 (3.24, 28.71)), and acute kidney injury (aOR: 3.74 (1.06, 13.20)) were all associated with increased inpatient mortality among SLE patients with ME (P < .05 for all). Between 2007 to 2019, ME occurred in 0.2% of SLE hospitalizations, with a 4.2% average inpatient mortality over the study period. Female sex, antiphospholipid syndrome, and stroke were most strongly associated with increased inpatient mortality.

Exploring the Complexities of Non-Bacterial Thrombotic Endocarditis: Highlights from Literature and Case Studies.

Non-bacterial thrombotic endocarditis (NBTE) is a form of non-infective endocarditis characterized by the deposition of sterile fibrin and platelets on cardiac valves. Even though some studies have identified important pathophysiological features, many aspects remain poorly understood. Given its wide availability, transthoracic echocardiography is typically the initial diagnostic approach to the patient. Additionally, recent technological advancements in transesophageal echocardiography, such as three-dimensional and multiplanar reconstruction analysis, have significantly improved diagnostic accuracy over time. By presenting our case series and performing a literature review, we focused on the main pathophysiologic, diagnostic, and therapeutic aspects of this rare but potentially life-threatening disease.

A Case of Re-Do Aortic Valve Replacement for Early Bioprosthetic Valve Dysfunction due to a Large Vegetation of Nonbacterial Thrombotic Endocarditis

A Case of Re-Do Aortic Valve Replacement for Early Bioprosthetic Valve Dysfunction due to a Large Vegetation of Nonbacterial Thrombotic Endocarditis

Marantic Endocarditis Associated with Metastatic Fibrolamellar Hepatocellular Carcinoma in a Young Adult: About a Case

We describe the case of a 21-year-old patient presenting with marantic endocarditis of the aortic valve occurring in the context of underlying metastatic fibrolamellar hepatocellular carcinoma. The patient was admitted due to a pulmonary embolism complicating deep vein thrombosis in the right lower limb. Upon admission, the patient was hemodynamically stable and afebrile, with a poor general condition. Physical examination revealed a painful swelling in the right lower limb extending up to the thigh, hepatomegaly suggestive of a tumor, and moderate ascites. Cardiac auscultation did not reveal any murmurs or abnormal sounds. Laboratory tests showed a non-specific inflammatory syndrome, and blood cultures were negative. The electrocardiogram indicated regular sinus tachycardia. Echocardiography revealed a mobile, hyperechoic mass located on the ventricular aspect of the aortic valve, without regurgitation or stenosis. The patient was started on heparin therapy. Unfortunately, the patient passed away at home two weeks later, and no autopsy was performed.

Noninfective Endocarditis in a Young Patient with Systemic Lupus Erythematosus

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.

Ischemic stroke from non-bacterial thrombotic endocarditis embolization in Li-Fraumeni syndrome: A case report

Ischemic stroke from non-bacterial thrombotic endocarditis embolization in Li-Fraumeni syndrome: A case report

Non-bacterial thrombotic endocarditis and pancreatic cancer: A literature review.

e24002 Background: Non-bacterial thrombotic endocarditis (NBTE) is a condition in which sterile vegetations are deposited on the cardiac valves, disrupting their function, and posing a risk for embolic stroke. It is a rare condition with rates in autopsy studies ranging from 0.9-1.6%1, and with some of the highest rates observed in patients with pancreatic cancer (PC).2 A review of PC-associated NBTE was conducted to improve our understanding and recognition of this rare but serious clinical entity. Methods: We performed a literature search in PubMed from database inception to November 2023 pairing keywords “Non-Bacterial Thrombotic Endocarditis”, “NBTE”, “Marantic Endocarditis”, “Sterile Endocarditis”, and “Libman-Sacks Endocarditis” with “Pancreatic Cancer”, “Adenocarcinoma of the Pancreas”, and “Neuroendocrine Pancreatic Cancer”. We also used the search term “Marantic Endocarditis associated with Pancreatic Cancer: A case series”. The database was filtered only for full text, English language, and human species. Results: We reviewed 17 studies and included 19 patients in this review. Diagnostic presentation included cerebral vascular accident (CVA) (n = 8), endocarditis (n = 3), or a thrombotic pathology such as deep venous thrombosis (DVT) or pulmonary embolism (PE) (n =4). NBTE (n = 12) was predominantly diagnosed before pancreatic cancer (n = 5). Echocardiography was positive for vegetations in most specified cases (14/15), blood cultures were negative in all specified cases (12/12), and abdominal imaging was consistent with pancreatic cancer in all specified cases (15/15). Metastatic disease was found in 13 out of 19 cases, with spread to liver (n = 13), lung (n = 2), and the adrenal glands (n = 1). Valvular localization identified mitral valve (n = 13), aortic valve (n = 7), and the tricuspid valve (n = 2). Treatment primarily focused on anticoagulation (n = 17), with low molecular weight heparin (n = 8) being the most common agent followed by unfractionated heparin (n = 6). 14 patients had expired by study publication, with 7 deaths occurring due to complications from pancreatic cancer and 2 occurring due to complications from NBTE. Conclusions: NBTE is a rare condition with non-specific presentation, with some of the highest rates seen in PC. Diagnosis of NBTE should prompt immediate treatment as per guidelines and investigation for an underlying malignancy. In PC, especially with metastatic disease, clinicians should maintain a low threshold to initiate an endocarditis work-up in the presence of neurologic symptoms or evidence of cardiac dysfunction. However, it is also important to note that 31% of NBTE cases occurred in the absence of metastatic PC, suggesting that even patients without advanced disease are at risk. Although NBTE associated with PC remains rare, given the severity of both conditions, clinicians should consider work-up for one disease process in the presence of the other.

Pancreatic cancer with multiple liver metastasis complicating multi organ infarcts from Marantic endocarditis and Trousseau&amp;apos;s syndrome

Background: Marantic endocarditis and Trousseau&amp;apos;s syndrome are historically linked with pancreatic cancer. The patient had catastrophic embolic events which caused multi organ infarct due to underlying advanced pancreatic cancer. Doctors should be aware of early signs and symptoms of pancreatic cancer and conduct necessary clinical assessments and investigations that can prevent any severe complications. Case Presentation: A middle-aged healthy gentleman who presented with left-sided weakness, slurred speech, left-sided sensory neglect, mild headache, and fever. CT head showed acute right-sided temporal ischemic changes with subsequent magnetic resonance imaging (MRI) head showed multiple infarcts in the brain. A transesophageal echocardiogram reported possible MV vegetation. MRI of the liver was done due to deranged LFTs which showed multiple liver metastasis with a primary mass in the tail of the pancreas. Left leg ultrasound Doppler showed a large left leg deep vein thrombosis involving the femoral vein as he complained of left leg pain for 2 months and went to GP with right leg pain 3 months ago which was treated as a right ankle sprain. The clinical events explained that most likely he developed Trousseau&amp;apos;s syndrome 3 months ago and the embolic phenomena were due to rare Marantic endocarditis (Non-bacterial thrombotic endocarditis) secondary to pancreatic cancer. Ultrasound-guided biopsy of liver metastasis was planned, but sadly the patient passed away the next day. Conclusion: The diagnosis of early pancreatic cancer with imaging poses a challenge sometimes, therefore the pancreatic protocol of CT or MRI is the first line of investigation. Moreover, the cause of unprovoked thrombo-embolism should be investigated to rule out any underlying malignancy.

Echocardiographic findings in cancer-associated non-bacterial thrombotic endocarditis. Clinical series of 111 patients from single institution.

Echocardiographic assessment of cancer-associated non-bacterial thrombotic endocarditis (Ca-NBTE) is limited to cases reports and small clinical series. Identifying heart valves abnormalities and its relation to embolic complications and cancer types. Manual review of echocardiographic images and medical records of Mayo Clinic patients (03/31/2002-06/30/2022) was performed. Ca-NBTE in 111 patients (mean age 63.2±9.7 years, 66.7% female) predominantly affected mitral valves (MV) (69), 56 aortic (AV), 8 tricuspid (TV) and rarely pulmonic (PV) (1). In 18 patients 2 valves were involved, 3 and 4 valves involvement in only a single patient each. Embolic complications were prevalent (n=102, 91.9%). Ca-NBTE affected MV more frequently the on upstream (atrial) (90% vs 49.3%) and TV downstream (ventricular) side (75% vs 37.5%). NBTE size (cm) varied significantly among valves, with TV hosting the largest masses (0.63-2.40 x 0.39-1.77), compared to MV [(0.11-1.81 x 0.11-1.62), (length p=0.001; width p=0.03)], and to AV [(0.20-2.70 x 0.11-1.51), (length p=0.001; width p=0.056)]; MV masses were borderline longer in systemic compared to cerebral emboli (p=0.057). Majority of MV (79.6%) and AV (69.6%) had thickened leaflets. NBTE lesions commonly affected closing margins (73.9%MV, 85.7% AV, and 62.5% of TV), but rarely commissures of MV (8.7%), yet fairly frequently of AV (41.1%). Five patients had severe regurgitation of MV and 5 AV. Ca-NBTE manifests mainly as thrombotic mobile masses attached to thickened MV and AV, with distinct variations in size based on valve type. Embolic destination but not cancer type is associated with NBTE mass size, and location.

Libman–Sachs endocarditis: regression after immunosuppressive therapy in a patient with systemic lupus erythematosus

Libman–Sachs endocarditis (nonbacterial thrombotic endocarditis, NBTE) may be one of the cardiac manifestations of systemic lupus erythematosus (SLE). It is characterized by the presence of sterile platelet thrombi on previously normal heart valves. The diagnosis of NBTE is difficult as it is often asymptomatic until complications such as systemic emboli or valvular dysfunction occur. Patients with Libman–Sachs endocarditis and SLE are treated with immunosuppressive and anticoagulant therapy and, if it is ineffective, surgical treatment.A clinical case of SLE and Libman–Sachs endocarditis is presented. The clinical manifestations, diagnostic methods and treatment of NBTE in SLE are discussed. The difficulties in differential diagnosis and selection of optimal treatment tactics faced by physicians are highlighted.

Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review

BackgroundNon-bacterial thrombotic endocarditis (NBTE) is a rare condition marked by sterile vegetations on cardiac valves, often linked to rheumatologic diseases, autoimmune disorders, and advanced solid malignancies. An early diagnosis and treatment of the associated clinical condition are mandatory, although they do not usually eliminate valvular vegetations, making anticoagulation essential to prevent embolic events. Despite variability, the prognosis of NBTE is usually unfavorable due to recurrent embolic events and the severity of the primary condition, typically advanced cancer.Case presentationWe present a case of a 57 years-old male who presented to the emergency department with a 5-day history of painful bilateral digital edema and color change episodes (from pallor to cyanosis). Physical examination revealed erythrocyanosis in the distal extremities, prompting consideration of secondary Raynaud syndrome.Despite medical therapy, progressive digital ischemia led to multiple finger amputations. During etiological investigation, anticoagulation tests and autoimmune analysis yielded negative results. A transesophageal echocardiogram was performed, revealing an irregular hyperechogenic mass on the anterior leaflet of the mitral valve without valve dysfunction, and a thoracic computed tomography scan with contrast showed an enlarged right paratracheal lymph node. Histopathological analysis from a transthoracic needle biopsy of the paratracheal lymph node revealed diffuse large B-cell lymphoma. The patient underwent aggressive R-CHOP chemotherapy, achieving a favorable complete response.ConclusionThis is a particular case involving the occurrence of NBTE and Raynaud phenomenon as the initial paraneoplastic manifestations in a previously healthy young man. Reports of NBTE associated with lymphoproliferative conditions are quite rare, with fewer than ten cases described in the literature. To our knowledge, this is the first case of NBTE specifically associated with diffuse large B-cell lymphoma.

Trousseau's syndrome with non-bacterial thrombotic endocarditis (NBTE) in a patient with advanced pancreatic cancer

Trousseau's syndrome with non-bacterial thrombotic endocarditis (NBTE) in a patient with advanced pancreatic cancer

Marantic Endocarditis in a Patient With a History of Remission From Cancer as a Cause of Systemic Embolism: A Case Report

Marantic Endocarditis in a Patient With a History of Remission From Cancer as a Cause of Systemic Embolism: A Case Report

PET-scan in diagnosis of non-bacterial thrombotic endocarditis: a case report

BackgroundNon-bacterial thrombotic endocarditis (NBTE), also known as marantic endocarditis or Libman–Sacks endocarditis, is a rare non-infectious condition affecting mostly the left-sided heart valves of patients who, most often, suffer from malignancies and connective tissue disorders. Herein, we present a case of a male patient with marantic endocarditis due to occult lung adenocarcinoma.Case presentationThe patient fulfilled the modified Duke’s criteria of possible bacterial endocarditis of the aortic valve; however, multiple blood cultures and serological tests were negative. In addition, the patient’s clinical course was constantly deteriorating. Thus, a fluorodeoxyglucose-positron emission tomography (FDG-PET) scan was conducted. This examination revealed multiple positive thoracic lymph node groups, as well as a positive lung lesion. The patient underwent aortic valve replacement and lymph biopsies during the operation established the diagnosis of solid/micropapillary lung adenocarcinoma and consequently of the non-bacterial thrombotic endocarditis.ConclusionsAdvanced imaging techniques may be needed to diagnose NBTE and should be kept in mind when the Duke criteria are not definite. Clinical suspicion is key to implement these premises. However, the exact role of the PET-scan has yet to be specified.

Cancer-associated non-bacterial thrombotic endocarditis-Clinical series from a single institution.

Premortem clinical presentation of cancer-associated non-bacterial thrombotic endocarditis (Ca-NBTE), therapy, and the clinal course is limited to case reports and small clinical series. An electronic search of Mayo Clinic records (03/31/2002-06/30/2022) with a subsequent manual review was performed to identify adult patients with echocardiographically detected NBTE and active malignancy, excluding those with infectious endocarditis or lupus anticoagulant/antiphospholipid antibodies. In this retrospective cohort study, we analyzed 115 Ca-NBTE patients (mean age 63.2 ± 9.7 years, 66.1% female) involving 71 (61.7%) mitral, 58 (50.4%) aortic, 8 (6.9%) tricuspid, and 1 (0.9%) pulmonary valve. The most common cancer was lung (n = 45 cases (39.1%), followed by pancreatic (n = 19, 16.5%), gynecological (17, 14.8%), gastrointestinal (n = 10, 8.7%), and 10 (8.7%) with hematologic malignancy; 6 patients had two active cancers. Embolic complications at presentation were frequent: 94 (81.7%) brain, 11 splenic, 10 renal, 6 coronary, and 4 to the extremities. Of 104 anticoagulated patients, 60 received low molecular weight heparin, 17 unfractionated heparin, 16 apixaban, 8 warfarin, and 3 rivaroxaban. There were 18 arterial thromboembolisms; the Kaplan-Meier estimates of the incidence at 2 years were consistent with a rate of 15.9% [95% Confidence Interval (CI) 9.9-23.3], including 14 strokes (12.4%, 95%CI, 7.1-19.2), and 8 other arterial emboli (10.5%, 95%CI, 4.7-18.9); there were 10 venous thromboembolisms (8.9%, 95%CI, 4.5-15.0). Fourteen major bleedings occurred (12.8%, 95%CI, 7.3-19.9) and 94 patients died during follow-up (77.9%, 95%CI, 71.1-85.8). Ca-NBTE predominantly affected women with lung adenocarcinoma or digestive tract cancers and manifested by stroke with high mortality and frequent embolic and bleeding complications during anticoagulation therapy.

Paraneoplastic Syndrome Associated Immune Complications: A Narrative Review of the Literature

Paraneoplastic syndromes occur in cancer patients due to an alteration in their immune response. There are several factors that may result in the occurrence of paraneoplastic syndrome, including the presence of abnormal cytokines that cause widespread effects throughout the body. The paraneoplastic syndrome may be present in patients before a diagnosis of cancer, and thus, understanding it is crucial as it will help achieve a timely diagnosis, which may aid in improving the chance of treatment. It is associated with several complications/presentations in patients. In this review, we will discuss several paraneoplastic syndrome associated complications, including hypercoagulable state, venous thromboembolism, arterial thromboembolism, thrombotic microangiopathy, disseminated intravascular coagulation, and malignancy associated non-bacterial thrombotic endocarditis. Although some are rare, it is vital for clinicians to have a knowledge of each to allow time for management.

Pancreatic cancer with multiple liver metastasis complicating multi organ infarcts from Marantic endocarditis and Trousseau’s syndrome

Background: Marantic endocarditis and Trousseau’s syndrome are historically linked with pancreatic cancer. The patient had catastrophic embolic events which caused multi organ infarct due to underlying advanced pancreatic cancer. Doctors should be aware of early signs and symptoms of pancreatic cancer and conduct necessary clinical assessments and investigations that can prevent any severe complications. Case Presentation: A middle-aged healthy gentleman who presented with left-sided weakness, slurred speech, left-sided sensory neglect, mild headache, and fever. CT head showed acute right-sided temporal ischemic changes with subsequent magnetic resonance imaging (MRI) head showed multiple infarcts in the brain. A transesophageal echocardiogram reported possible MV vegetation. MRI of the liver was done due to deranged LFTs which showed multiple liver metastasis with a primary mass in the tail of the pancreas. Left leg ultrasound Doppler showed a large left leg deep vein thrombosis involving the femoral vein as he complained of left leg pain for 2 months and went to GP with right leg pain 3 months ago which was treated as a right ankle sprain. The clinical events explained that most likely he developed Trousseau’s syndrome 3 months ago and the embolic phenomena were due to rare Marantic endocarditis (Non-bacterial thrombotic endocarditis) secondary to pancreatic cancer. Ultrasound-guided biopsy of liver metastasis was planned, but sadly the patient passed away the next day. Conclusion: The diagnosis of early pancreatic cancer with imaging poses a challenge sometimes, therefore the pancreatic protocol of CT or MRI is the first line of investigation. Moreover, the cause of unprovoked thrombo-embolism should be investigated to rule out any underlying malignancy.