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Background
This is characterizaed by the formation of a sterile platelet and fibrin thrombi on cardiac valves and adjacent endocardium. This usually occurs when the valve or heart tissue has been traumatized, or if there is a systemic state which predisposes to hypercoagulation. Unlike its infectious cousin, infective endocarditis, cultures of the vegetations are sterile.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Noninfective Endocarditis
Nonbacterial Thrombotic Endocarditis
Nonbacterial thrombotic endocarditis in a Japanese autopsy sample. A review of eighty cases.Chino F, Kodama A, Otake M, Dock DS.
Am Heart J 1975 Aug;90(2):190-8 Abstract quote A study of nonbacterial thrombotic endocarditis has been carried out in a series of 3,404 autopsies performed upon atomic bomb survivors in Hiroshima in the period 1953-1970.
The prevalence of the lesion was 2.4 per cent, with a greater frequency among the elderly and among females, and with a significant relationship to malignant neoplasms. In contrast to other reported series, there was a greater prevalence among cancers of the colon, rectum, and female genitourinary tract. No relationship was noted between the presence of NBTE and exposure to ionizing radiation. Histologic findings in the heart-valve leaflets in close proximity to the verrucae, like experimental studies reported by others, suggest that in association with severe systemic disease there appears a process consisting of degenerative changes in valve collagen and ground substance, with subsequent denudation of endothelium, localized almost entirely to the apposing leaflet surfaces of the left-heart valves. The verrucae of nonbacterial thrombotic endocarditis are then formed upon this abnormal leaflet surface. While the relationship between systemic disease and the pathologic changes observed in cardiac valve tissue is unclear, and although it is not known whether a "hypercoagulable state" may accentuate the tendency for thrombi to form upon these abnormal valves, there is no doubt that this lesion represents a clinically important complication of severe systemic disease. It also seems likely that in some cases
NBTE may complicate an illness which may otherwise be curable. Increasing awareness of this pathologic entity among clinicians, coupled with appropriate laboratory techniques, most likely echocardiography, will permit more frequent diagnosis in living patients.
DISEASE ASSOCIATIONS CHARACTERIZATION ADENOCARCINOMA
Mucin-producing adenocarcinomas and nonbacterial thrombotic endocarditis: pathogenetic role of tumor mucin.Min KW, Gyorkey F, Sato C.
Cancer 1980 May 1;45(9):2374-82 Abstract quote The clinicopathologic data of 5 patients who died of nonbacterial thrombotic endocarditis (NBTE) and disseminated thrombosis and multiple infarcts associated with mucin-producing adenocarcinomas were presented.
Histochemical studies on the valvular vegetations and thrombi revealed that histochemically stainable mucinous substance was an integral part. In 1 patient, circulating mucinous substance was found prior to the development of NBTE and disseminated thrombosis, and the identical mucinous substance was found in the tumor tissue, indicating a possible pathogenetic role of tumor mucin in NBTE-associated mucin-producing adenocarcinomas.
Our observations and reports of others suggest that intravascular mucin may be responsible for the clinical syndromes of mucinous malignancies, venous thrombosis, and NBTE with emboli.
BONE MARROW TRANSPLANT
Nonbacterial thrombotic endocarditis in bone marrow transplant patients.Patchell RA, White CL 3rd, Clark AW, Beschorner WE, Santos GW.
Cancer 1985 Feb 1;55(3):631-5 Abstract quote We reviewed the findings in 2381 consecutive autopsies, a series containing 91 bone marrow transplant (BMT) patients, and found 45 cases of nonbacterial thrombotic endocarditis (NBTE). The overall prevalence of NBTE was 1.9%. NBTE was present in seven BMT patients, for a prevalence of 7.7%. The rate of NBTE in BMT patients was significantly greater than the rate of NBTE in the general autopsy population (P less than 0.001).
The frequency of NBTE was not increased in patients with the individual underlying diseases for which BMT was a possible treatment (the BMT candidate diseases [BMTCD]). When rates of NBTE were compared in patients with BMTCD, there was a significantly higher rate in patients who had received BMT than in those who had not (P less than 0.02). These findings indicate that NBTE occurs with greater frequency in BMT patients and that there may be features of the BMT regimen that predispose patients to develop NBTE. Although several factors related to BMT are discussed as potential contributors to the increased prevalence of NBTE, we were not able to demonstrate an association between NBTE and any single factor.
Since NBTE is an important cause of arterial embolization, an awareness of the increased frequency of NBTE in BMT patients is important for clinicians who must interpret neurologic and other abnormalities in BMT recipients.
DIC
Nonbacterial thrombotic endocarditis (NBTE) and disseminated intravascular coagulation (DIC): autopsy study of 36 patients.Kim HS, Suzuki M, Lie JT, Titus JL.
Arch Pathol Lab Med 1977 Feb;101(2):65-8 Abstract quote A pathogenetic relationship between nonbacterial thrombotic endocarditis (NBTE) and disseminated intravascular coagulation (DIC) was sought by reviewing autopsies from a recent 12-year period. Of 4,783 autopsies, 36 patients were found to have NBTE. The histopathologic diagnosis of DIC in patients with NBTE was dependent on the observation of thrombi in vasa recta and central glomerular capillaries of the kidneys and in sinusoids, arterioles, capillaries, venules, and medium-sized veins of the major viscera.
Morphologic evidence of DIC was found in 18 (50%) of the 36 patients with NBTE. In addition, venous and arterial thromboses were found in 13 patients (36%) and pulmonary thromboembolism in 17 (47%).
These findings support the view that NBTE and DIC may be pathogenetically related and result from a hypercoagulable state.
GIANT CELL ARTERITIS Non-bacterial thrombotic (marantic) endocarditis associated with giant-cell arteritis.
Hesselink DA, van der Klooster JM, Schelfhout LJ, Scheffer MG.
Department of Internal Medicine, Medisch Centrum Rijnmond Zuid, Locatie Clara, Olympiaweg 350, 3078 HT, Rotterdam, The Netherlands
0953-6205 2001 Sep;12(5):454-458 Abstract quote A patient with giant-cell arteritis and non-bacterial thrombotic (marantic) endocarditis of the mitral valve is described.
To our knowledge, this is the first case reported. The importance of revising the diagnosis of infective endocarditis when no pathogen can be demonstrated is emphasized.
LUNG DISEASE
Association between nonbacterial thrombotic endocarditis and hypoxigenic pulmonary diseases.Truskinovsky AM, Hutchins GM.
Department of Pathology, Johns Hopkins Hospital, Pathology 401, 600 North Wolfe St., Baltimore, MD 21287, USA
Virchows Arch 2001 Apr;438(4):357-61 Abstract quote Observation of patients with nonbacterial thrombotic endocarditis (NBTE) in the setting of hypoxia from various lung diseases raised the question of a possible pathogenetic relationship between hypoxia and the development of NBTE.
We reviewed 50 autopsied patients with NBTE and compared them with 50 age/race/gender-matched control patients without NBTE. We noted the lung weight and graded the histopathological severity of lung involvement by disease, clinical respiratory compromise, and the extent of any cancer present. Patients with NBTE had heavier lungs (P < 0.01) and histologically and clinically more severe pulmonary disease (both P < 0.005). There was no statistically significant difference in the extent of metastatic cancer between the NBTE patients and the controls (P > 0.5).
When patients with cancer were excluded from the group of NBTE cases, there was still a statistically significant preponderance in the mean lung injury and clinical compromise scores of the NBTE patients (both P < 0.05), but the difference in lung weight was no longer statistically significant (P > 0.05). The study suggests that, in some patients, hypoxia may lead to NBTE, possibly through altered coagulation states.
PATHOGENESIS CHARACTERIZATION TISSUE FACTOR
Tissue factor is associated with the nonbacterial thrombotic endocarditis induced by a hypobaric hypoxic environment in rats.Nakanishi K, Tajima F, Nakata Y, Osada H, Ogata K, Kawai T, Torikata C, Suga T, Takishima K, Aurues T, Ikeda T.
Division of Environmental Medicine, National Defense Medical College Research Institute, Tokorozawa, Japan.
Virchows Arch 1998 Oct;433(4):375-9 Abstract quote High-altitude hypoxia causes a hypercoagulable state. In our previous study on the blood coagulation system in rats, nonbacterial thrombotic endocarditis (NBTE) developed after 4-12 weeks' exposure to the equivalent of 5500 m in altitude.
We hypothesized that TF (tissue factor)-producing cells in the cardiac valves might be induced by the hypobaric hypoxic environment (HHE) and then trigger NBTE. A total of 170 male Wistar rats were housed in a chamber at the equivalent of 5500 m altitude for 1-12 weeks. We measured TF activity in the plasma and studied morphological changes in the mitral valves using immunohistochemical and immunoelectrical methods for TF protein and in situ hybridization for TF mRNA. After 4 weeks or more of exposure to HHE, 28 of the 56 surviving rats had developed NBTE. After 4-8 weeks' exposure to HHE, the plasma TF activity level was significantly higher than in control rats. There was a significant correlation between plasma TF activity and the incidence of NBTE. After 1 weeks' exposure to HHE, immunoreactivity for TF protein was detected in foamy macrophages and stromal cells in the cardiac valves.
In rats with NBTE, TF protein was present in foamy macrophages and spindle stromal cells and focally present in the extracellular matrix. TF mRNA was detected in some foamy macrophages within the thrombus, TF protein was localized to the rough endoplasmic reticulum and plasma membrane of many macrophages, some fibroblasts, and a few endocardial cells. TF is associated with the pathogenesis of the NBTE induced by exposure to HHE. The accumulation of TF-producing macrophages during exposure to HHE may be responsible for initiating thrombus formation.
LABORATORY/RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC
Nonbacterial thrombotic endocarditis. Assessment by transesophageal echocardiography.Blanchard DG, Ross RS, Dittrich HC.
Department of Internal Medicine, University of California, San Diego School of Medicine.
Chest 1992 Sep;102(3):954-6 Abstract quote Nonbacterial thrombotic endocarditis (NBTE) is difficult to detect antemortem and is often not suspected until embolic events occur. Transthoracic echocardiography is useful in diagnosing NBTE, but it may be limited by suboptimal imaging and resolution.
Herein we describe the first reported case of NBTE diagnosed by transesophageal echocardiography. As early detection and treatment may avert significant embolic complications, transesophageal echocardiography should be strongly considered if other techniques are nondiagnostic and clinical suspicion of NBTE remains high.
Cardiac valvular vegetations in cancer patients: a prospective echocardiographic study of 200 patients.Edoute Y, Haim N, Rinkevich D, Brenner B, Reisner SA.
Department of Internal Medicine C, Rambam Medical Center, Haifa, Israel.
Am J Med 1997 Mar;102(3):252-8 Abstract quote PURPOSE: Nonbacterial thrombotic endocarditis can complicate various malignancies and may cause morbidity and mortality mainly as a result of systemic embolism. The antemortem diagnosis of nonbacterial thrombotic endocarditis is rare. The purpose of our study was to assess the frequency, echocardiographic characteristics, and clinical correlation of nonbacterial thrombotic endocarditis in cancer patients.
PATIENTS AND METHODS: A prospective echocardiographic screening of 200 nonselected ambulatory patients with solid tumors was performed. Patients were evaluated for evidence of thromboembolic events and for plasma D-dimer levels. A cohort of 100 consecutive patients without overt heart disease referred to echocardiography for the detection of an occult arterial embolic source served as a control group. It consisted of 52 males and 48 females, median age 60 years.
RESULTS: The study group included 87 women and 113 men, median age 64 years (range 21 to 91). The frequent malignancies were lymphoma (26%), carcinoma of the gastrointestinal tract (20%), and carcinoma of the lung (16%). Cardiac valvular vegetations were found in 38 patients (19%) compared with only in 2 patients in the control group (2%, P < 0.001). Vegetations were found on the mitral or on the aortic valve in 19 and 18 patients, respectively. Isolated tricuspid valve vegetation was found in 1 patient. Valvular lesions were mostly common in patients with carcinoma of the pancreas (3 of 6, 50%), carcinoma of the lung (9 of 32, 28%), and lymphoma (10 of 52, 19%). Thromboembolism was diagnosed in 22 (11%) patients (12 deep vein thrombosis, 4 emboli to extremities, 2 cerebrovascular accidents, and 4 "silent" segmental left ventricular wall motion abnormalities on echocardiography). Thromboembolism was noticed in 9 of 38 patients (24%) with vegetations compared with 13 of 162 patients without vegetations (8%; P = 0.013). Plasma D-dimer level was examined in a subgroup of 170 patients. D-dimer level was increased in 19 of 21 patients (90%) with thromboembolism compared with 76 of 149 patients without thromboembolism (51%; P = 0.001).
CONCLUSIONS: This study demonstrated a high prevalence of cardiac valvular lesions in patients with solid tumors. Vegetations were associated with thromboembolism. Plasma D-dimer level was significantly increased in patients with thromboembolism.
Echocardiography in nonbacterial thrombotic endocarditis: from autopsy to clinical entity.Reisner SA, Brenner B, Haim N, Edoute Y, Markiewicz W.
Department of Cardiology, Internal Medicine C, Rambam Medical Center, Haifa, Israel.
J Am Soc Echocardiogr 2000 Sep;13(9):876-81 Abstract quote Bacteria-free verrucae, frequently termed "non-bacterial thrombotic endocarditis," have been recognized in autoimmune disorders as well as in neo-plastic diseases.
The antemortem diagnosis of non-bacterial thrombotic endocarditis is rare, and most existing data result from postmortem examinations. In 3 prospective echocardiographic studies we found typical cardiac valvular lesions in patients with primary antiphospholipid syndrome, myelo-proliferative disorders, and solid malignant tumors.
Cardiac lesions associated with these 3 different entities had common echocardiographic appearance and correlated positively with thromboembolic events. The possibility of common pathogenesis is suggested, and clinical significance is discussed.
Acute ischemic stroke patterns in infective and nonbacterial thrombotic endocarditis: a diffusion-weighted magnetic resonance imaging study.Singhal AB, Topcuoglu MA, Buonanno FS.
Stroke Service, Department of Neurology, Massachusetts General Hospital, and Harvard Medical School, Boston, Mass 02114, USA
Stroke 2002 May;33(5):1267-73 Abstract quote BACKGROUND AND PURPOSE: 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).
METHODS: 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.
RESULTS: 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.
CONCLUSION: 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.
LABORATORY MARKERS
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL MARANTIC
Marantic endocarditis in children and young adults: clinical and pathological findings.Young RS, Zalneraitis EL.
Stroke 1981 Sep-Oct;12(5):635-9 Abstract quote The clinical and pathologic findings of 7 children and young adults with marantic endocarditis are reviewed. Cerebral embolic infarction attributable to the marantic vegetations occurred in 3 patients. The most common neurologic findings were altered mental status, seizures, and hemiplegia. Five of the 7 patients had had cardiac catheterization. Sepsis, pneumonia, hypoxia, disorders of coagulation, and renal failure were frequently present in these seriously ill patients.
In each instance, the diagnosis of marantic endocarditis was unsuspected and established only at autopsy.
VARIANTS NEONATAL
Neonatal nonbacterial thrombotic endocarditis.Krous HF.
Arch Pathol Lab Med 1979 Feb;103(2):76-8 Abstract quote Neonatal nonbacterial thrombotic endocarditis (NBTE), a rare disorder yet to be diagnosed antemortem, is described in two infants.
The first infant was postmature and suffered from polycythemia and meconium aspiration. The meconium-stained placenta manifested evidence of ischemia and disseminated intravascular coagulation (DIC). The second patient was delivered near term by cesarean section, and hyaline membrane disease developed.
The pathogenesis of NBTE may relate to perinatal hypoxia with transient tricuspid insufficiency, polycythemia, and DIC.
SKIN
Cutaneous manifestations of marantic endocarditis.Kimyai-Asadi A, Usman A, Milani F.
Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA.
Int J Dermatol 2000 Apr;39(4):290-2 Abstract quote A 70-year-old patient with a history of hypertension and hypercholesterolemia was referred for evaluation of necrotic toes. The patient had a history of several cerebrovascular accidents during the previous month. Initially, she developed sudden-onset left upper extremity weakness which, over the ensuing 4 days, progressed to complete left-sided weakness. This was followed by the development of acute dysarthria. A transesophageal echocardiogram revealed moderate left ventricular hypertrophy, several vegetations on her tri-leaflet aortic valve associated with moderate aortic regurgitation, and a large right atrial thrombus with a mobile component. Bubble studies failed to reveal any septal defects. The patient's electrocardiogram was nonspecific. As serial blood cultures were negative despite fevers of up to 39.8 degrees C, the patient was treated with a 6-week course of intravenous ceftriaxone, ampicillin, gentamicin, and ciprofloxacin for a presumed diagnosis of culture-negative endocarditis. Fungal cultures of the blood were negative.
The patient, however, progressed and developed several necrotic toes. Physical examination was significant for ischemic changes of the left first, second, third, and fifth toes, as well as the right first and second toes. Diffuse subungual splinter hemorrhages in the toenails, numerous 2-4-mm palpable purpuric papules on the lower extremities, and nontender hemorrhagic lesions of the soles were also noted. Peripheral and carotid pulses were intact and no carotid bruits were heard. Cardiopulmonary and abdominal examinations were unremarkable. Neurologic examination revealed a disoriented, dysarthric patient with left central facial nerve paralysis, as well as spasticity, hyperactive reflexes, and diminished strength and sensation in the left upper and lower extremities. A left visual field defect and left hemineglect were also present. The patient's last brain computerized tomogram revealed areas of low attenuation consistent with cerebral infarctions in three distinct areas of the brain. These included the left occipitotemporal area, the right parieto-occipital area, and the right posterior frontal region. The regions affected were in the distribution of both the anterior and posterior circulation. No evidence of hemorrhage was noted. The patient subsequently complained of abdominal discomfort. A computerized tomogram of the abdomen with oral and intravenous contrast revealed a 4-cm x 3-cm irregular mass in the tail of the pancreas with several low-attenuation lesions throughout the liver which were consistent with infarctions or metastases. Several splenic infarctions were also present. A biopsy of the tumor revealed pancreatic adenocarcinoma. The patient's carcinoembryonic antigen level was 18. 4 ng/mL (0-3) and the CA 19-9 antigen level was 207,000 U/mL (0-36). The alpha-fetoprotein level was normal. Other significant laboratory findings included a prothrombin time of 16.7 (international normalized ratio, 1.4), an activated partial thromboplastin time of 32 (ratio, 1.3), and a platelet count of 85,000/mm3.
The Russell viper venom time, sedimentation rate, and C3 levels were normal, and the patient was negative for antinuclear antibodies, anticardiolipin antibodies, and antibodies to extractable nuclear antigens. Of note, the patient was not receiving any anticoagulation. Blood cultures for mycobacteria and fungi, human immunodeficiency virus serology, and urinalysis and culture were negative. The patient subsequently developed an inferior wall myocardial infarction and was transferred to the coronary care unit. In line with the family's request, aggressive care was ceased and the patient expired. The patient's family refused an autopsy.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Surgical pathology of nonbacterial thrombotic endocarditis in 30 patients, 1985-2000.Eiken PW, Edwards WD, Tazelaar HD, McBane RD, Zehr KJ.
Mayo Medical School, Division of Anatomic Pathology, Mayo Clinic, Rochester, Minn. 55905, USA.
Mayo Clin Proc 2001 Dec;76(12):1204-12 Abstract quote OBJECTIVE: To describe the causes, complications, and histological appearance of nonbacterial thrombotic endocarditis (NBTE) in a surgical population compared with those in previously reported autopsy series.
PATIENTS AND METHODS: Cases were identified by reviewing the surgical pathology reports for all cardiac valvular specimens removed at Mayo Clinic, Rochester, Minn., between 1985 and 2000. Archived microscopic slides and medical records were reviewed for each study patient.
RESULTS: The study group consisted of 30 patients (20 female and 10 male), with a mean age of 49 years (range, 15-89 years). Of these 30 patients, 28 had single valve involvement (19 mitral, 8 aortic, and 1 tricuspid), and 2 had involvement of both their mitral and aortic valves. An underlying immune-mediated disorder was identified in 18 patients (60%), including primary antiphospholipid syndrome (in 8), rheumatic heart disease (in 6), systemic lupus erythematosus (in 2), and rheumatoid arthritis (in 2), 15 (83%) of whom were women. Of the remaining 12 patients with no autoimmune disease, only 5 (42%) were women. No patient had metastatic malignant disease or disseminated intravascular coagulopathy. Systemic embolization was documented in 10 patients (33%), 8 of whom had cerebral involvement. Valvular vegetations were visualized by echocardiography before surgery in 8 patients and were suspected but not confirmed preoperatively in 1 patient. All vegetations consisted primarily of platelets and fibrin. The site and appearance of vegetations did not vary with the underlying disease state.
CONCLUSIONS: In contrast to previously reported autopsy series, NBTE in a surgical population was more commonly associated with autoimmune disorders than malignancy or disseminated intravascular coagulopathy. Women were affected twice as often as men. Systemic embolization, particularly to the brain, was prominent in both surgical and autopsy series. Vegetations had a similar appearance regardless of the specific underlying disease. An antemortem diagnosis of NBTE in a patient with no known risk factors should prompt a search not only for occult malignancy, as suggested by autopsy studies, but also for autoimmune or rheumatic diseases, particularly the antiphospholipid syndrome.
SPECIAL STAINS/IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE
Immunohistological studies on valvular vegetations in nonbacterial thrombotic endocarditis (NBTE).Lehto VP, Stenman S, Somer T.
Acta Pathol Microbiol Immunol Scand [A] 1982 May;90(3):207-11 Abstract quote Thrombi and thrombus formation in nonbacterial thrombotic endocarditis (NBTE) were studied using light microscopy and immunohistology. Samples from vegetations on cardiac valves were taken at autopsy from five patients with NBTE and adenocarcinoma as an underlying disease. Morphological studies disclosed proliferative changes underneath the thrombi. In immunofluorescence microscopy, focal deposits of immunoglobulins and complement components Clq and C3 were found.
The results suggest that immune complexes, elicited by the underlying malignant process, may play important role in the pathogenesis of the thrombus formation in NBTE.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS STROKE
Multiple cerebral infarctions from nonbacterial thrombotic endocarditis mimicking cerebral vasculitis.Vassallo R, Remstein ED, Parisi JE, Huston J 3rd, Brown RD Jr.
Thoracic Diseases Research Unit, Division of Pulmonary and Critical Care Medicine and Internal Medicine, Mayo Clinic Rochester, Minn. 55905, USA.
Mayo Clin Proc 1999 Aug;74(8):798-802 Abstract quote Primary vasculitis of the central nervous system (PVCNS) is an uncommon disorder that can present with a variety of symptoms, making diagnosis and management difficult.
We describe a case of cerebral infarction that occurred from nonbacterial thrombotic endocarditis (NBTE) and presented with clinical and radiologic imaging features that suggested PVCNS. The patient was a 58-year-old woman with left hemiparesis, aphasia, and episodic confusion. Magnetic resonance imaging of the brain demonstrated multifocal lesions consistent with infarction involving both cerebral hemispheres, and cerebral angiography showed changes consistent with vasculitis. Although brain biopsy findings were normal, the patient was treated for presumed vasculitis with cyclophosphamide and prednisone. Four months later respiratory failure secondary to polymicrobial pneumonia and adult respiratory distress syndrome developed, and she died. Autopsy revealed multiple infarcts in the heart, lungs, right kidney, spleen, and brain. Multiple thrombotic platelet-fibrin vegetations consistent with NBTE were found on all cardiac valves. Examination of the brain revealed no evidence of active or healed vasculitis.
Cerebral angiography may show findings that suggest vasculitis, but it is not diagnostic, as several other conditions may cause similar changes. Nonbacterial thrombotic endocarditis may cause multiple cerebral infarctions and can be difficult to distinguish from vasculitis, as specific diagnostic tests for PVCNS are lacking.
Fatal cerebroembolism from nonbacterial thrombotic endocarditis in a trauma patient: case report and review.Sharma S, Mayberry JC, Deloughery TG, Mullins RJ.
Department of Surgery, Oregon Health Sciences University, Portland 97201-3098, USA.
Mil Med 2000 Jan;165(1):83-5 Abstract quote Nonbacterial thrombotic endocarditis (NBTE) is a rare condition that may result in an unexpected and usually fatal cerebroembolism. It occurs in a variety of clinical situations, including malignancy, immune disorders, and sepsis, but it has rarely been reported after trauma.
The formation of NBTE appears to require a hypercoagulable state and changes in valvular morphology, e.g., during a hyperdynamic state. Patients with disseminated intravascular coagulation have a 21% incidence of NBTE. Although NBTE is usually found at autopsy, premorbid detection by echocardiography is currently possible and feasible. Untreated patients have a high incidence of embolic events, but anticoagulation with heparin may be life-saving. A lethal case of NBTE in a severely injured patient is reported here with the purpose of increasing awareness among medical personnel caring for trauma patients.
Recommendations have been made for surveillance echocardiography in high-risk patients, e.g., critically ill patients with sepsis or disseminated intravascular coagulation.
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Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.
Last Updated 7/5/2002
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