Multiple pulmonary embolism in patients with ventriculoatrial shunts: a report on two cases

Pulmonary embolism occurred in two patients with a Spitz-Holter shunt. One patient had a massive pulmonary embolism which resulted in the development of pulmonary hypertension. The patient suffered another episode of embolism during warfarin therapy and died. Autopsy revealed a massive belateral pulmonary embolism. No thrombi were found in the venous system. The other patient had experienced symptoms that suggested recurrent pulmonary embolism before the established diagnosis. Thereafter she has constantly been treated with oral anticoagulant therapy without the recurrence of emboli.     

The optimal duration of anticoagulant treatment following pulmonary embolism

The optimal course of oral anticoagulant therapy is determined according to the risk of recurrent venous thromboembolism after stopping therapy and the risk of anticoagulant-related bleeding. Clinical risk factors appear to be important in predicting the risk of recurrence whereas the influence of biochemical and morphological tests is uncertain. The risk of recurrent venous thromboembolism is low when the initial episode was provoked by a reversible major risk factor (surgery): 3 months of anticoagulation is sufficient. Conversely, the risk is high when venous thromboembolism was unprovoked or associated with persistent risk factor (cancer): 6 months or more prolonged anticoagulation is necessary. After this first estimation, the duration of anticoagulation may be modulated according to the presence or absence of certain additional risk factors (major thrombophilia, chronic pulmonary hypertension, massive pulmonary embolism): 6 months if pulmonary embolism was provoked and 12 to 24 months if pulmonary embolism was unprovoked. If the risk of anticoagulant-related bleeding is high, the duration of anticoagulation should be shortened (3 months if pulmonary embolism was provoked and 3 to 6 months if it was unprovoked). Lastly, if pulmonary embolism occurred in association with cancer, anticoagulation should be conducted for 6 months or more if the cancer is active or treatment is on going. Despite an increasing knowledge of the risk factors for recurrent venous thromboembolism, a number of issues remain unresolved. Randomised trials comparing different durations of anticoagulation are needed.     

Deep vein thrombosis and pulmonary embolism: prevention, diagnosis, and treatment.

Pulmonary embolism (PE) is a significant cause of mortality in the elderly. More than 90% of pulmonary emboli originate from a thrombus in the deep veins of the legs. Proper diagnosis and treatment of deep vein thrombosis (DVT) are thus essential to prevent PE. Diagnosis of new or recurrent DVT is based on the results of one or more tests, including impedance plethysmography (IPG) or duplex venous scan; venography can often be avoided, based on results of initial testing. For suspected PE, perfusion lung scanning is the initial test of choice, followed by IPG/duplex or venography. Pulmonary angiography is indicated for patients with decreased cardiorespiratory reserve. Decisions governing prophylaxis of DVT are based on individual relative risk; prophylactic therapies include intermittent compression, low-dose heparin, and oral anticoagulants. Management of thromboembolism requires IV and oral anticoagulant therapy.     

Partial anomalous pulmonary venous return: case report and review of the literature.

Isolated partial anomalous pulmonary venous return (APVR) is an uncommon finding. A patient with isolated APVR had pulmonary hypertension without demonstrable left-to-right shunting prior to anticoagulant treatment of pulmonary emboli. After anticoagulant therapy, with a fall in pulmonary pressures and resistance to near-normal levels, left-to-right shunting was then detected by oximetry and angiography. The anomaly was visualized on electron beam angiography and confirmed by conventional angiography after anticoagulant therapy. Contrary to the expected obligatory drainage of highly saturated blood associated with APVR, lack of detectable shunting was thought to be due to the obstruction of regional blood due to thromboembolism.     

Management of pulmonary embolism

Venous thromboembolism is often triggered by transient episodes of increased risk but may occur spontaneously in patients with permanently increased risk. Pulmonary embolism may cause impairment of respiratory and circulatory function which can vary in severity from silent to catastrophic. The diagnosis can be feasibly established by detecting vein thrombosis. If this approach fails, demonstration of pulmonary emboli by lung scans becomes necessary to establish the diagnosis. A D-dimer-test can select patients in whom imaging of pulmonary perfusion is required. Immediate therapeutic anticoagulation with heparins is mandatory in confirmed thromboembolism, and is followed by a vitamin K antagonist. In the initial work-up, patients also have to be evaluated for systemic thrombolytic therapy by hemodynamic parameters and echocardiography. In a triggered episode of venous thromboembolism, duration of anticoagulation is confined to several months while spontaneous or recurrent events require prolonged or indefinite treatment.     

Pulmonary thromboendarterectomy for chronic pulmonary thromboembolism in three patients with systemic lupus erythematosus and antiphospholipid syndrome]

Pulmonary hypertension (PH) is a serious and often fatal complication of systemic lupus erythematosus (SLE). Several potential mechanisms have been postulated for narrowing of vessels as a result of pulmonary vasculitis and pulmonary thromboembolism caused by antiphospholipid antibodies. Pulmonary thromboendarterectomy for chronic pulmonary thromboembolism is performed to alleviate pulmonary hypertension. We report three rare cases of SLE with antiphospholipid syndrome in patients who presented with PH secondary to chronic pulmonary thromboembolism. Pulmonary thromboendarterectomy was performed, and all patients remained well without deterioration of PH after surgery. Pulmonary thromboendarterectomy should be considered as an effective method of treatment for this disease.     

Unilateral chronic thromboembolic pulmonary disease associated with combined inherited thrombophilia.

Chronic thromboembolic pulmonary hypertension (CTEPH) is considered to be an extreme variant of pulmonary thromboembolism. The underlying mechanisms for the failure of thrombus resolution are still unclear. In looking for inherited thrombophilia, an association with a lupus anticoagulant has been described repeatedly, and single cases of anticoagulant deficiencies (ie, antithrombin [AT], protein C, and protein S) have been reported. We describe a young patient with type I AT deficiency, the heterozygous prothrombin G20210A mutation, and unilateral chronic thromboembolic pulmonary disease presenting after a single thrombotic event. Pulmonary vascular patency was restored successfully by surgical pulmonary thromboendarterectomy. This case is unique because unilateral CTEPH is extremely uncommon, and it illustrates the severe clinical sequelae of the cosegregation of inherited thrombophilic defects.     

Pulmonary Tumor Thrombotic Microangiopathy from Metastatic Gallbladder Carcinoma: An Unusual Cause of Severe Pulmonary Hypertension

Pulmonary hypertension and cor pulmonale due to tumor emboli causing pulmonary tumor thrombotic microangiopathy (PTTM) is rare and extremely difficult diagnosis to make prior to death. Pulmonary hypertension due to metastatic tumor emboli should be included in the differential diagnosis of various causes of dyspnea in patients with a history of cancer or more common causes, including infection, thromboembolism, metastasis, adverse effects of drugs, and recurrent effusions. We describe a patient with gallbladder carcinoma who presented with progressive dyspnea and severe pulmonary hypertension. The etiology was tumor emboli and PTTM from gallbladder carcinoma, which remained elusive prior to her death despite appropriate clinical investigations and was established on autopsy. To the best of our knowledge, this is likely the second reported case of PTTM from metastatic gallbladder carcinoma.     

Pulmonary hypertension from chronic pulmonary thromboembolism

Pulmonary hypertension may develop whenever chronic obstruction of pulmonary arterial blood flow occurs. Although repeated pulmonary embolism is thought to be the usual underlying cause, there is little clinical evidence to support this theory. Studies of the pulmonary vascular endothelium have shown that perturbations of the normal endothelium can create a procoagulant environment, which could lead to the development of thrombosis in situ at the level of the large or smaller pulmonary vessels. Some patients develop proximal pulmonary thromboemboli, which may be the result of retrograde propagation of thrombus after an initial pulmonary embolus. Others present with unexplained pulmonary hypertension secondary to thrombotic occlusion of the pulmonary microvasculature. A perfusion lung scan will show abnormalities that should lead to correct clinical diagnosis and confirmatory evaluation. Thromboendarterectomy in selected cases provides dramatic clinical improvement in patients with proximal thromboemboli. Vasodilators may be effective in some patients with obstruction at the arteriolar level. Both groups should be treated with chronic warfarin anticoagulant therapy to protect against progression of thromboembolism.     

A case of primary antiphospholipid syndrome complicated with pulmonary hypertension]

We describe the case of 55-year old male with antiphospholipid syndrome (APS) who developed pulmonary hypertension without any thromboembolic episode. Multiple pulmonary perfusion defects suggestive of in situ thrombosis were observed. Hematological findings revealed microangiopathic hemolytic anemia and thrombocytopenia. These findings were improved by anticoagulant therapy. We monitored mean pressure of pulmonary artery (mPAP) and total pulmonary vascular resistance (TPR) before and after using vasodilator agents by Swan-Ganz catheter. mPAP and TPR showed improvement on treatment with oxygen supplementation therapy and Isosorbide administration. Previously 11 cases with APS complicated with pulmonary hypertension were reported. Majority of these patients have had recurrent venous thrombosis, particularly deep vein thrombosis often accompanied by pulmonary thromboembolism (8/11 cases, 72%). However in this case pulmonary hypertension with APS may be induced by in situ thrombosis in pulmonary micro vessels.     

肺动脉肉瘤误诊为肺血栓栓塞症三例原因分析

目的　提高对肺动脉肉瘤的认识及正确诊断率。方法　结合 3例肺动脉肉瘤患者的临床资料和有关文献对肺动脉肉瘤及肺血栓栓塞症 (PTE)的临床特点进行对比分析。结果　肺动脉肉瘤与肺血栓栓塞症临床表现相似 ,但肺动脉肉瘤患者发病隐匿 ,多有发热、体重减轻 ,无下肢深静脉血栓形成病史 ,影像学显示单侧肺动脉扩张 ,主肺动脉及左、右肺动脉内大量肿块 ,临床上经溶栓和抗凝治疗后病情仍恶化 ,确诊需手术。结论　肺动脉肉瘤易误诊为PTE ,需引起临床注意     

A new noninvasive management strategy for patients with suspected pulmonary embolism

Pulmonary embolism is associated strongly with proximal-vein thrombosis. We tested the hypothesis that recurrent venous thromboembolism is unlikely in the absence of proximal-vein thrombosis. We performed a prospective cohort study in 874 patients with suspected pulmonary embolism. On long-term follow-up of 371 patients with the following characteristics: (1) abnormal, non-high-probability lung scans, (2) off anticoagulant therapy, and (3) serial noninvasive test results negative for proximal-vein thrombosis, only 10 (2.7%; 95% confidence limits, 1.3% to 4.9%) had venous thromboembolism. By comparison, venous thromboembolism on follow-up occurred in 3 (1%) of 315 patients (95% confidence limits, 0.2% to 2.8%) with normal lung scans, and in 5 (7%) of 66 patients (95% confidence limits, 2.5% to 16.8%) with high-probability lung scans. Patients whose serial noninvasive test results were negative for proximal-vein thrombosis have a good prognosis without anticoagulant therapy.     

Prognosis in pulmonary embolism

Acute pulmonary embolism has a wide prognostic spectrum, ranging from sudden death within minutes of a thromboembolic episode to a benign treatable condition associated with a stable clinical course and no long-term sequelae. In patients who survive an initial thromboembolic episode and receive antithrombotic therapy, the clinical course can be complicated by recurrent nonfatal venous thromboembolism, fatal pulmonary embolism, the postthrombotic syndrome, and chronic thromboembolic pulmonary hypertension. Identifying which patients are at increased risk of experiencing these sequelae is important in decision making relating to the aggressiveness of initial antithrombotic therapy, the duration of antithrombotic therapy, and the frequency of clinical surveillance. In addition, this information may be helpful to clinicians in discussing disease prognosis with patients. The objectives of this review are to provide reasonable estimates of the risks of recurrent nonfatal venous thromboembolism, fatal pulmonary embolism, the postthrombotic syndrome, and chronic thromboembolic pulmonary hypertension in patients with treated pulmonary embolism, and to identify risk factors for these sequelae.     

A case of pulmonary thromboembolism due to circulating lupus anticoagulant

A 22-year-old man was admitted because of hemosputum and progressive dyspnea with 3 attacks of chest pain and dyspnea over the previous 4 months. Chest roentgenography showed pulmonary infarction of the left lower lobe, and the diagnosis was confirmed by pulmonary perfusion and inhalation scintigraphy and pulmonary arteriography. Thrombolytic therapy was performed, but no significant effect could be obtained and anticoagulant therapy was performed continuously. No deep-vein thrombosis could be seen. He was considered to have autoimmune hemolytic anemia with lupus anticoagulant on the basis of auto-antibody data. Lupus anticoagulant is an antibody to phospholipid, and it is suggested that a decrease in the production of prostanoid in the endothelium causes thrombosis. In this case, as the patient showed a low level of 6-keto-PGF1 alpha in the blood, it is suggested that one of the etiological factors of pulmonary thromboembolism is a disorder of prostacyclin production in the endothelium, causing thrombosis by lupus anticoagulant.     

Pulmonary infarction complicating thrombophlebitis of the upper extremity

Five cases of thrombophlebitis of the upper extremity in which pulmonary embolism occurred as a complication are presented. The etiology was secondary to chemical irritation in two cases, was unknown in one case and was probably traumatic in the fifth case. All of the patients having pulmonary emboli received anticoagulant therapy. Pulmonary emboli were controlled satisfactorily in two of the five patients. One patient died from repeated pulmonary emboli originating in the right axillary, brachial and subclavian veins, and multiple pulmonary infarctions were demonstrated at postmortem examination. Venous ligation was necessary in one of the remaining cases, and the fifth patient continued to have hemoptysis for six weeks even though the prothrombin time was maintained at therapeutic levels. This patient subsequently had recurrent thrombophlebitis of the upper extremity with pulmonary embolism, from which he recovered with conservative therapy.     

Pulmonary hypertension, systemic lupus erythematosus, and the contraceptive pill.

Pulmonary hypertension in systemic lupus erythematosus (SLE) in the absence of chronic parenchymal lung disease or pulmonary emboli is rare. We report such a case with an acute and rapidly progressive onset of symptoms in a patient who had started taking the contraceptive pill eight months previously.     

Management der Lungenembolie

Venous thromboembolism is often triggered by transient episodes of increased risk but may occur spontaneously in patients with permanently increased risk. Pulmonary embolism may cause impairment of respiratory and circulatory function which can vary in severity from silent to catastrophic. The diagnosis can be feasibly established by detecting vein thrombosis. If this approach fails, demonstration of pulmonary emboli by lung scans becomes necessary to establish the diagnosis. A D-dimer-test can select patients in whom imaging of pulmonary perfusion is required. Immediate therapeutic anticoagulation with heparins is mandatory in confirmed thromboembolism, and is followed by a vitamin K antagonist. In the initial work-up, patients also have to be evaluated for systemic thrombolytic therapy by hemodynamic parameters and echocardiography. In a triggered episode of venous thromboembolism, duration of anticoagulation is confined to several months while spontaneous or recurrent events require prolonged or indefinite treatment.     

Chronic pulmonary embolism and pulmonary hypertension

Incomplete resolution of acute pulmonary embolism (PE) is frequently observed after acute PE and may rarely result in chronic thromboembolic pulmonary hypertension (CTEPH). The underlying pathophysiological mechanism is largely unknown. Evidence underlines the concept of a dual pulmonary vascular compartment model consisting of increased pulmonary vascular resistance by both large vessel obstruction and distal small vessel obliteration, the latter initiated by pathological vascular remodeling. Up to 40% of patients with established CTEPH have no prior history of symptomatic venous thromboembolism. CTEPH is associated with a poor prognosis if left untreated. Therefore, the diagnostic approach of CTEPH aims at assessing the location and extent of the embolic obstruction, establishing the operability and prognosis of the patients and ruling out other variations of pulmonary hypertension with distinct indicated treatment. Heart catheterization for invasive pressure measurements and pulmonary catheter angiography is obligatory for the final diagnosis. Pulmonary thromboendarterectomy is the treatment of choice. In certain patients with persistent or recurrent pulmonary hypertension after surgery or with inoperable disease, pharmacotherapy might be beneficial.     

原发性肺动脉肉瘤——附一例报告

目的 提高对原发性肺动脉肉瘤的认识。方法 通过一肺动脉肉瘤病例,详细分析此类疾病的临床症状、放射学特征及病理表现。结果 本病与其他肺动脉阻塞性疾病（包括肺血栓栓塞）的临床表现并无明显差异。对疑为肺血栓栓塞的病人,当放射影像学出现单侧肺动脉扩张、持续性软组织充盈的肺动脉、肺内肿块,临床呈现经抗凝和溶栓治疗后病情依然恶化等情况时,均高度提示本病。结论 肺动脉肉瘤极易误诊为其他肺血管阻塞性病变,应予重视     

A rare cause of pulmonary-renal syndrome with autoantibodies: primary antiphospholipid syndrome

We present a case of primary antiphospholipid syndrome with acute renal failure and alveolar haemorrhage. He was successfully treated with cyclophosphamide, corticosteroids and plasma exchange. Patients with antiphospholipid syndrome may develop a broad spectrum of pulmonary disease. Pulmonary thromboembolism and pulmonary hypertension are the most common complications, but alveolar haemorrhage have also been reported. Other causes need to be excluded. Despite favourable outcome of many patients with association of plasma exchange, immunosuppressive drugs and anticoagulant therapy, definite conclusions about the best therapeutic regimen could not be draw.