Nonbacterial Thrombotic Endocarditis Research Articles

Non-bacterial Thrombotic Endocarditis (NBTE) in the Absence of Malignancy or Lupus Anticoagulant/Antiphospholipid Antibodies: A Case Report

Non-bacterial Thrombotic Endocarditis (NBTE) in the Absence of Malignancy or Lupus Anticoagulant/Antiphospholipid Antibodies: A Case Report

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.

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.

Nonbacterial Thrombotic Endocarditis Presenting as a Mass in the Right Atrium Diagnosed by Open Chest Surgery: A Case Report.

Nonbacterial thrombotic endocarditis (NBTE) is a condition that results in the development of vegetation on cardiac valves that are devoid of inflammation and bacteria. We herein report a 60-year-old man who transferred to our hospital because of a systemic embolism and heart failure. A mass in the right atrium and vegetation on the mitral valve were observed. He was first diagnosed with infectious endocarditis according to the Duke criteria. During treatment, however, the patient was diagnosed with antiphospholipid syndrome and cancer. After four weeks of antibacterial therapy, the patient underwent open chest surgery, and the postoperative histological diagnosis was NBTE.

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 assessment and investigations which 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 ischaemic changes with subsequent MRI head showed multiple infarcts in the brain. Transoesophageal echocardiogram reported possible MV vegetation. MRI of the liver was done due to deranged LFTs which showed multiple liver metastasis with primary mass in the tail of pancreas. Left leg ultrasound doppler showed a large left leg deep vein thrombosis involving 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 right ankle sprain. The clinical events explained that most likely he developed Trousseau's syndrome 3 months ago and the embolic phenomena was 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 investigation. Moreover, the cause of unprovoked thrombo-embolism should be investigated to rule out any underlying malignancy.

Abstract 16013: Non-Bacterial Thrombotic Endocarditis and Recurrent Cerebrovascular Accidents in Immune Checkpoint Inhibitor Myocarditis

Background: Nonbacterial thrombotic endocarditis (NBTE) is a rare condition associated with cancer, hypercoagulable state, and systemic emboli including stroke. This report presents a case of NBTE associated with metastatic lung adenocarcinoma and recurrent strokes despite anticoagulation for prior pulmonary embolism (PE) and highlights the challenges in management of NBTE. Case: 66-year-old man with stage IV non-small cell lung adenocarcinoma treated with pembrolizumab complicated by immune checkpoint inhibitor (ICI) myocarditis treated with high dose steroids and PE treated with rivaroxaban, presented with diplopia and gait instability. MRI brain revealed a left corona radiata infarct and rivaroxaban was switched to apixaban. Three weeks later, the patient developed left internal carotid artery (ICA) occlusion, and underwent emergent thrombectomy. A transesophageal echocardiogram revealed a new 1.8 cm mass on the mitral valve with negative blood cultures. Anticoagulation was switched to therapeutic enoxaparin. Cardiothoracic surgery was consulted, though patient opted for palliative care. Decision making: Patient presented with recurrent multifocal strokes despite treatment with multiple direct oral anticoagulants (DOACs), just weeks after ICI myocarditis. The challenges here were approach to anticoagulation in a patient with recurrent strokes despite anticoagulation, the risk of hemorrhagic conversion of stroke, and the mechanism of stroke. The mitral valve mass was presumed to be NBTE, due to his metastatic malignancy and negative blood cultures. However, a recent history of ICI-myocarditis raised the possibility of an immune mediated mechanism of NBTE, given the growing association of auto-immune diseases and NBTE. Conclusion: NBTE is a rare complication of metastatic disease that can commonly lead to stroke and is treated with optimal anticoagulation and addressing the underlying malignancy. However, this case highlights the failure of multiple oral anticoagulants to treat NBTE and raises the question of additional mechanisms of NBTE beyond malignancy and hypercoagulable state. Further studies are required to elucidate a common immune mediated mechanism of NBTE and ICI myocarditis.

Abstract 14774: Differences in the In-Patient Mortality in Marantic Endocarditis Per Etiology: Systematic Review of Case Reports

Introduction: Non-bacterial thrombotic endocarditis (NBTE; also known as marantic endocarditis) is a unique entity characterized by sterile fibrin deposits on valves caused by hypercoagulability and endocardial injury. Literature is limited regarding the distinction between its causes based on in-hospital mortality. Hypothesis: Would a significant difference in in-patient mortality be present in NBTE admissions depending on etiology? Methods: A review of the literature using the PubMed, Embase, Cochrane, and Web of Science databases was conducted to obtain case reports and series reporting NBTE from inception to September 2022. Crude and univariate analyses were performed. Results: 396 case reports and 20 case series were identified, of which 450 patients were extracted. Females represented 64% of our sample (median age: 46, interquartile range [IQR]: 32 - 60) while 36% were males (median age: 50, IQR: 36 - 64). Overall all-cause in-hospital mortality was 36%. NBTE was associated with cancer in 202 (47%), autoimmune disease in 191 (45%), and other causes in 34 (8%) cases. Older population and cancer etiology were associated with higher mortality (OR: 1.77, CI 95%: 1.19 - 2.62 & OR: 8.29, CI 95%: 4.97 - 13.83, respectively). A significant decrease in mortality is noted in the more recent years compared to the 20 th century (OR: 0.10, 95% CI: 0.06 - 0.18). Conclusions: Although an improvement in all-cause in-hospital mortality for admission related to NBTE has been noticed in recent years, cancer as an etiology and old age are two risk factors associated with higher mortality.

Abstract 16055: Marantic Endocarditis - A Rare Paraneoplastic Presentation of Ovarian Carcinoma

Introduction: Nonbacterial thrombotic endocarditis (NBTE) or Marantic endocarditis, involves formation of sterile platelet & fibrin thrombi on undamaged cardiac valves & adjacent endocardium. The vegetation is associated with embolization. Case presentation: A middle-aged female without cardiac history presented to the ED with blurred vision & fatigue for 3 days. The patient was afebrile with a BP of 183/97mmHg. Physical examination showed decreased visual acuity in the left eye. The EKG was normal. The patient underwent a CT scan brain that showed suspicion of bilateral occipital strokes. MRI brain revealed multiple small acute infarcts in the bilateral cerebral hemispheres & cerebellum. Blood cultures were negative. Telemetry did not show atrial fibrillation. Given a suspicion of cardioembolic stroke, the patient underwent a transesophageal echocardiogram (TEE) which demonstrated a trileaflet aortic valve (AV) with abnormal irregular thickening of leaflets, concerning for vegetation & moderate-severe aortic regurgitation (AR). figure 1. Our patient was diagnosed with marantic endocarditis based on TEE & serial negative cultures. Apixaban was started. Further workup of the patient revealed bilateral pulmonary emboli. Abdominal imaging revealed a pelvic mass, ultimately diagnosed as clear-cell ovarian cancer. Interval TEE at 3 months demonstrated complete resolution of vegetations with preserved cusp motion & mild AR. A shared decision was made to continue lifelong anticoagulation. Discussion: NBTE commonly involves mitral & AV. Risk factors include trauma, circulating immune complexes, hypercoagulability, malignancy & acute inflammation. Symptoms result from embolization, valve dysfunction, or impaired cardiac function. It is based on imaging after ruling out infectious etiologies. Management is anticoagulation & valve replacement with the treatment of the underlying etiology. Valve replacement prevents future embolic complications.

Abstract 18687: Cardio-Rheumatology: Coronary and Cerebrovascular Thromboses in Antiphospholipid Antibody Syndrome

Description of Case: A 27-year-old male with antiphospholipid antibody syndrome (APLS) with a high-risk antibody profile presented for cardiac evaluation. APLS was diagnosed after presentation with acute cardioembolic stroke requiring thrombolysis. Echocardiography showed a normal ejection fraction, with multiple small echo-densities attached to the anterior and posterior leaflet tips of the mitral valve suggesting non-bacterial thrombotic endocarditis (NBTE). The patient was treated with warfarin and aspirin was recommended but declined. As the patient reported lightheadedness, a cardiac rhythm monitor was placed and showed two episodes of non-sustained ventricular tachycardia (NSVT). A cardiac MRI was then obtained, showing multifocal, subendocardial and mesocardial delayed gadolinium enhancement suggesting fibrosis, in a distribution most consistent with a multivessel embolic event. A CT coronary angiogram was performed to further evaluate the coronary arteries, revealing normal coronary anatomy, free from atherosclerotic plaque or thrombus. Beta-blocker therapy was initiated for arrhythmic episodes, with resolution of symptoms and no recurrence of NSVT on follow-up monitoring. Left ventricular (LV) function continued to remain preserved on follow-up imaging, with no progression of mitral valve vegetations on anticoagulation. Discussion: Cardiac involvement in APLS has been well described but largely centers around valvular disease and de novo venous and arterial thromboses. This case of cerebrovascular and coronary pathology in APLS as a likely consequence of thromboembolic phenomena is an unusual presentation and highlights the potential downstream consequences of left-sided valvular NBTE. A high index of suspicion is required to specifically evaluate for myocardial scarring despite normal LV function on echocardiography. The presence of myocardial fibrosis in these cases can act as a focus for scar-related ventricular arrhythmias, which if diagnosed and treated in a timely manner can limit further cardiovascular complications for the patient.

Abstract 18878: Right Heart Valves Affected by Cancer-Related Non-Bacterial Thrombotic Endocarditis as Potential Cause of Pulmonary Embolism

Background: Ca-NBTE primarily affects the mitral valve (MV) and aortic valve (AV), with rare involvement of the right heart valves. Premortem descriptions of this condition are limited to a few case reports. This study presents an initial clinical series focusing on patients with Ca-NBTE specifically affecting their right heart valves. We describe the underlying cancers, types of valve lesions, and associated embolic complications. Patients and Methods: A electronic search of medical records (March 31, 2002, to June 30, 2022) was followed by a manual review to identify adult patients with echocardiographically detected NBTE, active cancer, and no infectious endocarditis or lupus anticoagulant/antiphospholipid antibodies. Results: Out of 115 identified patients with Ca-NBTE (mean age 63.2±9.7 years, 66.1% female), there were 8 cases (7.0%) affecting right heart valves (mean age 64.5±12.1 years, 3 females) with underlying cancer: pancreatic (n=3), lung (n= 2), genitourinary (n=1), gastric (n=1) and neck sarcomatoid carcinoma (n=1). TV was affected in 8 patients and PV in one case; 4 patients had Ca-NBTE limited only to PV, 2 patients had MV and TV, 1 patient had mitral, aortic and TV, and 1 patient with pancreatic cancer had all four valves affected by NBTE. 5 patients had embolic complications to the brain with one patient in that group also experiencing emboli to the kidney and spleen. Out of 4 patients with NBTE limited to TV, two still had brain emboli, one had only pulmonary embolism, and one patient had no embolic complications. Conclusion: Around 7% of Ca-NBTE cases involve the right heart valves, with half of these cases limited to the TV. The cancer profile in these cases resembles that seen in left heart valve involvement.

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

Multi-valve Libman-Sacks's endocarditis-related multiple, massive and fatal systemic embolization. A case report and a review of diagnostic work-up.

We reported a case of non-bacterial thrombotic endocarditis (NBTE) in a 37-year-old woman who presented with signs and symptoms of cardio-embolic cerebral stroke caused by a prothrombotic state due to underlying advanced uterine cancer. Multimodal imaging, including 3D-ecocardiography, as well as laboratory and cultural tests, were critical in making the diagnosis. After starting anticoagulation therapy with low molecular weight heparin (LMWH), the patient underwent surgical aortic valve replacement due to worsening aortic valve function, initial left ventricle enlargement, increasing dimensions, and mobility of vegetations. Unfortunately, vegetations relapsed on the aortic valve bio-prosthesis as well as the mitral leaflets, resulting in a final picture of multi-valve NBTE. The fatal outcome was due to a massive multiple limb embolism, which resulted in leg amputations and septical complications. Starting with the case, we present a brief overview of the pathology's presentation, treatment, management, and prognosis, as well as the diagnostic work-up.

Towards a new role for multimodality imaging in the diagnosis of cancer-associated marantic endocarditis

Towards a new role for multimodality imaging in the diagnosis of cancer-associated marantic endocarditis

S2058 Marantic Endocarditis in Case of Newly Diagnosed Advanced Pancreatic Neoplasm

S2058 Marantic Endocarditis in Case of Newly Diagnosed Advanced Pancreatic Neoplasm

Libman–Sacks Vegetation, Infective Vegetation, and Pulmonic Valve Abscess in a Systemic Lupus Erythematosus Patient on Immunosuppressive Therapy

Abstract 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.