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- W2895064378 abstract "Case PresentationA 62-year-old woman with no medical history was admitted to the ED for fever, acute respiratory failure, and pain in the right lower limb. Three months prior to presentation, she had spent 45 days travelling through India and Thailand. She presented with no signs of traveler’s disease such as fever or diarrhea during that trip. Two weeks following her return to France, the patient presented with an episode of bronchitis and received 5 days of treatment with cefixime (a third-generation cephalosporin). Despite this antibiotic treatment, her symptoms worsened and she developed, over the following 3 weeks, general weakness and arthralgia/myalgia. Due to the severity of hypoxemia, the patient was immediately transferred to the ICU. A 62-year-old woman with no medical history was admitted to the ED for fever, acute respiratory failure, and pain in the right lower limb. Three months prior to presentation, she had spent 45 days travelling through India and Thailand. She presented with no signs of traveler’s disease such as fever or diarrhea during that trip. Two weeks following her return to France, the patient presented with an episode of bronchitis and received 5 days of treatment with cefixime (a third-generation cephalosporin). Despite this antibiotic treatment, her symptoms worsened and she developed, over the following 3 weeks, general weakness and arthralgia/myalgia. Due to the severity of hypoxemia, the patient was immediately transferred to the ICU. The patient’s hemodynamic examination revealed a heart rate of 115 beats/min and BP of 112/35 mm Hg. Her temperature was 38.5°C. Cardiac auscultation revealed a regular rhythm and a grade III/IV diastolic murmur. Her respiratory rate was 28 breaths/min, and her oxygen saturation was 96%, with 50% oxygen inspired fraction and 50 L/min gas flow. Urinary catheterization revealed oliguria with a urine output < 0.3 mL/kg/h over the first 4 h. Her abdomen was soft and not distended. The clinical examination revealed a cold, pale, and pulseless lower right limb, and the neurologic examination was significant for decreased cutaneous sensitivity in the same limb. The patient’s chest radiograph revealed bilateral pulmonary edema and right-sided pleural effusion (Fig 1). Transthoracic echocardiogram showed thickening of the aortic cusps (Fig 2A), preserved biventricular morphology and function, mild pericardial effusion, and bilateral pleural effusions. The color Doppler examination showed severe aortic regurgitation (Fig 2B). Transesophageal echocardiography revealed a tricommissural aortic valve, with small vegetations on the three cusps (Video 1) and confirmed the severity of the aortic regurgitation (Video 2). CT angiography showed the presence of a thrombus in a preexisting right external iliac artery stenosis and confirmed the diagnosis of acute right lower limb ischemia (Fig 3).Figure 2Long-axis parasternal view with transthoracic echocardiography revealed (A) thickening of the aortic cusps and (B) severe aortic regurgitation with color Doppler imaging.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Three-dimensional reconstruction of CT angiography (left anterior oblique view) showing the site of thrombosis in the right external iliac artery. Calcified aortic bifurcation and common iliac arteries are shown. Residual perfusion of the right femoral artery results from a branch of the internal iliac artery.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Due to the severity of the patient’s heart failure, urgent cardiac surgery involving an aortic valve replacement with a bioprosthesis was performed. Surgical inspection of the aortic valve revealed an unusual thrombotic aspect. Simultaneously, a femorofemoral crossover bypass graft was performed to restore right lower limb perfusion, and curative anticoagulation was started with IV unfractionated heparin. Results of all blood cultures since the patient’s hospital admission were negative. Moreover, results of all microbiologic investigations, including serology and serum polymerase chain reaction specific for rare causes of blood culture-negative infective endocarditis, were also negative. Pathologic examination of the resected aortic valve identified a preserved valvular structure and a thrombotic lesion, with aggregated platelets surrounded by fibrin (Fig 4). After stopping sedation, the patient woke up with complete right hemiplegia. The cerebral CT angiography revealed thrombosis of the left middle and right posterior cerebral arteries, with signs of established cerebral infarcts. Low hemoglobin levels (9.3 g/dL) and thrombocytopenia (73,000 per mm3) were found on the postoperative hemogram. Activated partial thromboplastin time was 81 s (normal value: 23-38 s), and the international normalized ratio was 1.43 (normal value: 0.9-1.2). Results of the anti-PF4 antibody test were negative. Autoimmune disorders were excluded by negative anti-nuclear, anti-double-stranded DNA and anti-neutrophil cytoplasmic antibodies. Immunologic assays for antiphospholipid syndrome, including IgG and IgM of anticardiolipin and anti-β2GP1 antibodies, were also negative. No pathologic activation of complement was found (normal CH50). What is the diagnosis? What is the most likely etiology in this case? Diagnosis: Nonbacterial thrombotic endocarditis. Most likely etiology: Paraneoplastic. Nonbacterial thrombotic endocarditis (NBTE) is secondary to a fibrin and platelet deposit on otherwise normal cardiac valves. It is frequently associated with malignancies (marantic endocarditis) but can be encountered in patients with autoimmune disease (Libman-Sacks endocarditis), severe burns, septicemia, and AIDS. Lung, pancreatic, and gastric cancers (particularly adenocarcinomas) are the most common neoplasms associated with NBTE. An advanced metastatic cancer is often involved. Clinically, systemic arterial thromboembolism, rather than valvular dysfunction, represents the main manifestation of the disease. Cerebral or cardiac emboli lead to the most severe morbidities. Compared with infective endocarditis, a higher risk of emboli has been described in NBTE. Because of the absence of a local inflammatory response, the thrombotic vegetations are more friable and prone to migrate. In the presence of fever, the differential diagnosis beyond infective endocarditis is difficult and requires a high level of clinical suspicion. NBTE should be suspected in patients with a known predisposing disease, in case of blood culture-negative endocarditis without a known predisposing disease, or in the absence of a response to antibiotic treatment, even when bacterial endocarditis was suspected. Multiple blood cultures and complete microbiologic investigations are required to rule out infective endocarditis. There are no specific echocardiographic findings that can confidently allow one to differentiate infectious vegetation from noninfectious vegetation. However, vegetations in NBTE are frequently small, rounded, sessile, broad based, and are often found on both sides of the heart. Transesophageal echocardiography has higher sensitivity (90%) for diagnosing vegetations than transthoracic echocardiography, particularly for vegetations that are < 5 mm. Pathologic analysis of the vegetation confirms the diagnosis of NBTE, with a preserved valve structure surrounded by fibrin deposits. The treatment of NBTE involves treating the underlying disease in association with anticoagulation. The risk of hemorrhagic conversion of embolic events must be evaluated, and a brain CT scan should be performed prior to anticoagulation. Long-term anticoagulation is necessary because recurrent systemic emboli occur in 50% of patients. Unfractionated heparin is the preferred anticoagulation drug. Warfarin is not indicated in patients with malignancy-associated NBTE, as persistent thromboembolic events while taking warfarin have been reported. Additional research is required to study the effects of direct thrombin and factor Xa inhibitors. There are no guidelines for surgical treatment of NBTE. Valvular surgery should be avoided as much as possible because the inflammatory response to on-pump cardiac surgery exacerbates the prothrombotic state and leads to a high risk of perioperative thromboembolic events. Surgical treatment (valvular repair or replacement) is reserved for cases of severe valvular dysfunction or persistent embolism despite anticoagulation. In the context of NBTE associated with cancer, once the malignancy has been identified, oncologic therapy remains the primary form of treatment. Without treatment of the underlying cancer, the prognosis associated with paraneoplastic NBTE is very poor. An elevated cancer antigen 125 level of 709 U/mL (normal value: < 35 U/mL) was recorded in the patient. No tumors were discovered via radiography, upper GI endoscopy, pelvic examination and radiography, or breast ultrasound. Empiric bone marrow aspiration found no infiltration by abnormal cells. 18F-fluorodeoxyglucose PET/CT imaging revealed abnormal hypermetabolic lesions in the retrocardiac area of the right lung (Fig 5A) and in the left subclavian lymph node area (Fig 5B). These results were consistent with an advanced cancer. After 4 weeks, the patient was still experiencing poststroke mutism, severe cortical blindness, and complete right hemiplegia. Taking into account the neurologic sequelae, the advance directives of the patient, and the wishes of her family, no additional investigation was done to confirm a pathologic diagnosis of underlying malignancy. The patient was discharged from the ICU, and hospital care at home was arranged. She died a few weeks later. 1.NBTE is the most common cause of valvular vegetations in blood culture-negative patients with advanced cancer or autoimmune disease.2.There is no specific echocardiographic pattern for NBTE. Given that vegetations are often small, transesophageal echocardiography is the preferred confirmatory diagnostic test.3.Recurrent systemic emboli occur in 50% of patients. Long-term antithrombotic treatment with unfractionated heparin is indicated.4.Surgical treatment of NBTE is reserved for severe symptomatic sequelae. It should be avoided whenever possible because it may exacerbate the underlying prothrombotic state.5.In cancer-related NBTE, diagnosing the underlying malignancy is critical because anticancer therapy is the mainstay of treatment. Financial/nonfinancial disclosure: None declared. Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiIzNTZkNmRhODRhZTEwMDI5Njc2NjgwYTY4YzhmODBkNiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NjY4OTAyfQ.aSw1GF0aUBGjPTZHEh8n_lhVNM_4natWxckc7dkjrhQD3x9Gp3_HAExcYRIm6BDB_iiEtrMbhzD3aWfFL6IlNNHx9JynWk2LsnKsnG90BsMGFwhbYhbHCHXZdT0TVVf-4lCZ7luwc2uyFGaqdbRi3Q8j1jra6w9fLeaoHECshh2Fhncymf5zDsZnE_uG6UKKBe3fkvOsCM_abNVSKSoaneMDdXlsOr9ta1ljZogjpiJwOmzGC1mr1Hzvsb4Slf7nGmskwMXvwGCj2awJ6l9PJsLSoS_dQ8ghWkczjrmpZG8TEHCLJbNxBpCBtoVJSGy1FJSRYGi3UnONfWN9ZrAajA Download .mp4 (0.62 MB) Help with .mp4 files Video 1Mid-esophageal short-axis view of the aortic valve showing the vegetation aspect on the three cusps.eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJjYzEzYmEzMGVlZDUxZTg1OWQ0Nzk0OTFhM2Q2YjliYiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NjY4OTAyfQ.eFcLiRTicrKyokKefbWSgH3ZXZELLX62I-tXkCRcgAH7V_72Ngv8UQ_jfGc8Ga9GSPhMKi4ng1IhgivvsrGbST5MU4wbCIekaJtYn-7We9LoqOy42njL70_sl14w45_tREOC_4l9XVj9KLfgumgfCb_XgizI8xjOzN60LA1g-oYEuNTEsN4A9IkSfRpxuR0WdLsaU4FM-wkv8tQd5D3wT3hjhHAHYihoYbYwAONzByh-LyvkHlaqDoxA_gLwy0Zr4PPOc7NxkFQa7bD2ekkxOCfQlYzozY6ZpeMhGAGyQkIJCQLrZPXmb2ChqMc_1IBCNkMiivc7s6IKQr2rk3VD9w Download .mp4 (0.72 MB) Help with .mp4 files Video 2Mid-esophageal long-axis view of the aortic valve with color Doppler imaging showing a severe aortic regurgitation." @default.
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- W2895064378 date "2018-10-01" @default.
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- W2895064378 title "A 62-Year-Old Woman With Acute Respiratory Failure and a Painful Right Lower Limb" @default.
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