Sterile Vegetations in Malignancy: A Rare Case of Nonbacterial Thrombotic Endocarditis in a Patient with Metastatic Melanoma.

Recurrent Embolism in the Course of Marantic Endocarditis

PV304 / #276Case Report Poster Topic:AS23 - SLE-Diagnosis, Manifestations, & OutcomesIntroductionLibman-Sacks endocarditis (LSE), a form of nonbacterial thrombotic endocarditis (NBTE), is characterized by fibrinous, sterile vegetations, favoring the mitral and aortic valves. LSE is observed in up to 11% of patients with systemic lupus erythematosus (SLE) as 1 of 2 major valvular manifestations, the other being thickening, with both mechanisms contributing to valvular dysfunction. LSE may typically be clinically silent, but predisposes to greater risk of cerebrovascular disease, underpinning the importance of its recognition. Complicating this, LSE may be clinically mimicked by infectious endocarditis; both may present with arterial embolism and may share elevated inflammatory markers and positive autoantibodies. Here, we present a complex first presentation of SLE with LSE resulting in cerebrovascular disease in a young patient. This case reinforces the importance of trans-esophageal echocardiography (TEE) in differentiating morphological features of NBTE from infectious endocarditis to guide timely management.Case Presentation With InvestigationA 22-year-old male of fit-and-well background presented with progressive lower limb swelling and right upper limb weakness. Broad-spectrum intravenous antibiotics were commenced for suspected infectious endocarditis, given trans-thoracic echocardiography (TTE) findings of moderate mitral regurgitation, magnetic resonance imaging (MRI) brain evidence of multiple embolic strokes (Figure 1), and progressive kidney injury with microscopic hematuria. Characteristic findings of LSE unseen on TTE were visualized by TEE: sessile posterior mitral valve leaflet tip lesions and thickening of the leaflets (Figure 2). This supported the clinical suspicion of NBTE, reinforced by persistent culture negativity. Laboratory tests confirmed positive antinuclear and double-stranded DNA antibodies, compatible with a first presentation of SLE, featuring cerebrovascular disease, LSE and lupus nephritis. Early TEE findings supported multidisciplinary team agreement to switch from intravenous antibiotics to high-dose corticosteroids.Figure 1.Figure 2.Literature ReviewLSE is an under-reported manifestation of SLE and is 1 of 2 mechanisms whereby lupus may affect the heart valves, alongside valvular thickening. SLE patients with neurological sequelae and high index of suspicion for NBTE should first undergo TTE to assess for valvular thickening or vegetations, as their presence relates to 3-fold greater numbers of circulating cerebral microemboli and subsequent cerebrovascular disease.[1] Trans-thoracic imaging, however, underestimates lupus-associated valve disease, and poorly differentiates NBTE from infective endocarditis. No individual biochemical test can confirm LSE; imaging must therefore play a vital role in differentiation. Importantly, 3-dimensional TEE possesses greater sensitivity and specificity than TTE and 2-dimensional TEE for visualization of LSE vegetations.[2] LSE-related valvular masses may be differentiated from bacterial vegetations based on their appearance, mobility, and location. Libman-Sacks vegetations are sessile, typically smaller (<10mm), are heterogeneous in echotexture, may show central calcification, and are visualized at the leaflet base. Conversely, bacterial lesions are homogenous in echotexture, are found at the line of leaflet closure, and move independently from the motion of the valve.[3] In our patient, we observed typical features of NBTE, namely valvular thickening (Figure 2a) and multiple small, sessile lesions (Figure 2b) correlating with his extracardiac manifestations of lupus including cerebrovascular disease (Figure 1).DiscussionOur case highlights the importance of trans-esophageal echocardiography in the diagnosis of SLE-related LSE and differentiation from infective endocarditis, which may mimic NBTE clinically and biochemically. TEE may complement trans-thoracic imaging in diagnosing NBTE to identify a subset of patients with SLE at greatest risk of cerebrovascular disease who may benefit from intensive immunosuppression.

Diagnosing Endocarditis: Get the Picture?!

We report a case of nonbacterial thrombotic endocarditis (NBTE) in a patient with bladder cancer presenting with multiple cerebral infarctions. Initial transthoracic and transesophageal echocardiography did not show any abnormalities. However, repeat transthoracic and transesophageal echocardiography demonstrated a vegetation on the anterior leaflet of the mitral valve with mild mitral regurgitation and no evidence of leaflet destruction. Persistent high-grade fevers and leukocytosis were observed. The patient was suspected to have infective endocarditis. However, abdominal ultrasound and computed tomography scan revealed multiple metastatic masses, and serial blood cultures were negative. The patient was ultimately diagnosed with NBTE associated with multiple metastases of bladder cancer. This case suggests that even if echocardiography does not initially demonstrate any abnormalities in patients with embolism, it must be repeated at the recurrence of embolism, and that even if clinical signs of infection are documented, NBTE should be suspected in any cancer patient with thromboembolic events.

Aortic Valve Vegetation Without Endocarditis

28-Year-Old Man With Recurrent Vertigo, Syncope, and Progressive Memory Impairment

Nonbacterial thrombotic endocarditis (NBTE) is illustrated by thrombi deposition on normal heart valves without the presence of bacteremia. It typically occurs in the setting of chronic debilitating diseases such as cancer or autoimmune disease. The pathogenesis involves an endothelial injury in the presence of a hypercoagulable state secondary to the effects of circulatory cytokines, which triggers platelet deposition. It usually forms on the upstream atrial surface of the mitral and tricuspid valves and the ventricular surface of the pulmonic and aortic valves and occurs most commonly in the fourth to eighth decades of life with no specific gender predisposition. These vegetations have a distinct morphology that varies from infective endocarditis (IE). Cerebrovascular lesions due to NBTE have a distinctive pattern of multiple, widely distributed small and large strokes on brain magnetic resonance imaging (MRI). We present a case of a 78-year-old man who was initially diagnosed as pneumonia and IE; he underwent a trans-esophageal echocardiogram (TEE), which revealed Libman-Sacks findings that have changed his diagnosis to lung cancer. We aim to highlight the characteristic TEE findings of NBTE to help clinicians search for underlying etiologies, including malignancies if NBTE is suspected.

A fifty-four-year-old woman died from multiple brain infarction and hemorrhage in the bilateral cerebrum, cerebellum, and brainstem, with renal infarction. She developed hematuria and transient blindness sixteen days before admission. Low-grade fever, heart murmur, and aortic valve vegetation on ultrasonic cardiography suggested infectious endocarditis. Autopsy study revealed occult adenocarcinoma in the lung and nonbacterial thrombotic endocarditis, but infective endocarditis was not histologically confirmed. The patient was considered to be a rare case of nonbacterial thrombotic endocarditis who developed multiple small infarctions mainly in the brainstem and cerebellum. Nonbacterial thrombotic endocarditis seems to be still an important disease as the embolic source, even if cryptic, of systemic thromboembolism.

Although infective endocarditis (IE) and nonbacterial thrombotic endocarditis (NBTE) are associated with cardioembolic stroke, differences in the nature of these conditions may result in differences in associated stroke patterns. We compared patterns of acute and recurrent ischemic stroke in IE and NBTE, using diffusion-weighted MRI (DWI). Using ICD-9 diagnostic codes and medical record review, we identified 362 patients (387 episodes) with IE and 14 patients with NBTE. Thirty-five patients (with 27 episodes of IE, 9 NBTE) who underwent 36 initial and 29 follow-up DWI scans were selected for this study. DWI lesion size, number, and location were compared between groups and correlated with stroke syndromes and endocarditis features. DWI was abnormal in all but 2 patients. Four acute stroke patterns were identified: (1) single lesion, (2) territorial infarction, (3) disseminated punctate lesions, and (4) numerous small (<10 mm) and medium (10 to 30 mm) or large (>30 mm) lesions in multiple territories. All patients with NBTE exhibited pattern 4, whereas those with IE exhibited patterns 1, 2, 3, and 4 (6, 2, 8 and 9 episodes, respectively). Seventy-five percent of patients with pattern 3 exhibited the clinical syndrome of embolic encephalopathy. Vegetation size, valve, and organisms had no correlation with stroke patterns. DWI has utility in differentiating between IE and NBTE. Patients with NBTE uniformly have multiple, widely distributed, small and large strokes, whereas patients with IE exhibit a panoply of stroke patterns.

Mitral Valve Aspergilloma in an Immunocompromised Patient with Recurrent Cerebrovascular Accidents

Staphylococcus hyicus, a novel pathogen causing destructive infective endocarditis requiring mitral annular reconstruction.

Objective To enhance understanding on echocardiographic and clinical characteristics of valve lesions of non-infective endocarditis (NIE), particularly in patients with systemic lupus erythematosus (SLE). Comparative analysis of the diagnostic value of echocardiography was performed in patients with non-infective endocarditis and atypical infective endocarditis (IE). Methods Data from 38 patients with clinically diagnosed NIE in the institution were collected retrospectively during July 2005 and January 2015, including 10 patients with SLE, 10 with rheumatic heart disease, 11 with rheumatoid arthritis, and 7 with hepatitis B. Data of 42 patients diagnosed as atypical IE during the same period were collected as control group. All patients underwent examinations of blood culture, sero-immunological tests, electrocardiogram and echocardiography. Comparison was made between the two groups using SPSS 11.5 software package. Results The difference in blood culture, sero-immunological tests and electrocardiogram was statistically significant between the groups (χ2 value, 26.29, 5.53, and 4.80, respectively, all P 0.05). Echocardiography identified valvular vegetations in 27 of 38 patients, with NIE with a detection rate of 71.0%; The size of the vegetations ranged from 2 to 7 mm in diameter; Valve vegetations was found in 36 of 42 patients with atypical IE, with a detection rate of 85.7%; the other six cases demonstrated valvular thickening only; in this group, the vegetations ranged from 2 mm to 19 mm in size and were located in the left heart in 28 patients, 8 cases in the right heart. In the case group, two cases of valve lesions in patients with SLE were confirmed by transesophageal echocardiography (TEE), while missed on TEE examination. Nine cases with more than mild valve regurgitation were identified. Ten cases were treated with hormones and cyclophosphamide, after which valve lesions resolution was found on serial echocardiography tests with a follow-up period of 5 days to 3 years. Conclusions Echocardiography is capable of detecting valve lesions at early stage in patients with NIE, particularly in patients with SLE. Echocardiography plays a crucial role in identifying the non-infective thrombotic vegetations, guiding clinical treatment and monitoring the therapeutic effects. Key words: Echocardiography; Endocarditis; Lupus erythematosus, systemic

LIBMAN-SACKS ENDOCARDITIS AS THE INITIAL PRESENTATION OF METASTATIC GASTRIC ADENOCARCINOMA

Nonbacterial thrombotic endocarditis in multiple heart valves.

We describe the case of a 43-year-old woman with transient ischemic neurologic deficits and recurrent systemic and pulmonary emboli in whom infectious work-up and extensive thrombophilic evaluation were unremarkable. Transesophageal echocardiography (TEE) established the diagnosis of nonbacterial thrombotic endocarditis (NBTE). This is a rare condition often associated with hypercoagulable states or advanced malignancy such as adenocarcinomas, characterized by cardiac vegetations along valvular coaptation lines without destruction of leaflets. In our patient, we diagnosed an ovarian clear cell adenocarcinoma, a malignant disorder that has been rarely reported in association with NBTE. This case illustrates that NBTE can present as an atypical manifestation of malignancy and must be distinguished from infective endocarditis, which implies a different therapeutic strategy. When confronted with findings of NBTE without a clear etiology, an occult neoplasm must be excluded. Anticoagulant therapy is the mainstay of treatment. However, cardiac vegetations may require surgical intervention in rare instances.