18-Fluorodeoxyglucose positron emission tomography can detect the pulmonary involvement of intravascular lymphoma that presents no abnormality in a computed tomography scan.
We report the case of a 61-year-old Japanese man who had pulmonary intravascular lymphoma and no computed tomography abnormality. We were able to make an antemortem diagnosis of pulmonary intravascular lymphoma by transbronchial lung biopsy according to 18-fluorodeoxyglucose positron emission tomography findings. He is free of recurrent disease 24 months after chemotherapy.
To the best of our knowledge, this is the first reported case of a long-term survivor of pulmonary intravascular lymphoma diagnosed by transbronchial lung biopsy under the guide of 18-fluorodeoxyglucose positron emission tomography.
NO SINGLE NONINVASIVE TEST for pulmonary embolism is both sensitive and specific. Some tests are good for “ruling in” pulmonary embolism (e.g., helical CT) and some tests are good for “ruling out” pulmonary embolism (e.g., D-dimer); others are able to do both but are often nondiagnostic (e.g., ventilation–perfusion lung scanning). For optimal efficiency, choice of the initial diagnostic test should be guided by clinical assessment of the probability of pulmonary embolism and by patient characteristics that may influence test accuracy. This selective approach to testing enables pulmonary embolism to be diagnosed or excluded in a minimum number of steps. However, even with the appropriate use of combinations of noninvasive tests, it is often not possible to definitively diagnose or exclude pulmonary embolism at initial presentation. Most of these patients can be managed safely without treatment or pulmonary angiography by repeating ultrasound testing of the proximal veins after one and 2 weeks to detect evolving deep vein thrombosis. Helical CT and MRI are rapidly improving as diagnostic tests for pulmonary embolism and are expected to become central to its evaluation.
Plasma concentrations of cross linked fibrin degradation products, a marker of intravascular thrombosis and fibrinolysis, were measured in 495 patients with suspected pulmonary embolism referred for ventilation-perfusion lung scanning to determine whether concentrations are increased in pulmonary embolism and their potential use in diagnosis. Lung scans were described as normal (n = 66) or as showing a low (n = 292), indeterminate (n = 58), or high probability (n = 79) of pulmonary embolism. There was a difference between the mean levels of cross linked fibrin degradation products in each scan category: normal scans, 142 ng/ml; low probability scans, 295 ng/ml; indeterminate probability scans, 510 ng/ml; high probability scans, 952 ng/ml (p less than 0.001). Of the patients with high probability scans, 96% had raised concentrations. Explanations for discrepant low results include incorrect scan diagnosis, delay in blood sampling, and anticoagulation. Of the patients with a low or indeterminate probability of pulmonary embolism, 43% had increased concentrations of cross linked fibrin degradation products that could be attributed in most cases to another illness. Owing to the wide range of values in each lung scan diagnostic category, raised concentrations of these fibrin degradation products cannot be used without reference to the patient's clinical state as a discriminatory test for pulmonary embolism. Further evaluation of the significance of normal concentrations in excluding a diagnosis of pulmonary embolism appears to be warranted.
Ventilation and perfusion lung scans were performed preoperatively and postoperatively in 169 patients and classified blindly according to preset criteria. Perfusion lung scan abnormalities were present in 25 (15%) of the preoperative scans and 42 (25%) of the postoperative scans; 16 (38%) of the 42 abnormal postoperative scans were identical to the preoperative scans. Perfusion defects indicating a "high probability" of pulmonary embolism (lobar or segmental defects) were present in 5 preoperative scans and 10 postoperative scans; the 10 postoperative scans were classified as showing "definite" (5), "possible" (1) or "no" (4) pulmonary embolism on the basis of the preoperative scan and the ventilation scan; none of the 10 patients had clinical evidence of pulmonary embolism. Venous thrombosis was present in 12 patients, including 4 of the patients whose lung scans showed definite pulmonary embolism. Thus, postoperative perfusion lung scan defects are potentially misleading even when large.
Acute pulmonary embolism (PE) is a common and often fatal disease with a mortality rate of more than 30% in untreated patients. There is a twofold increase in mortality in patients with massive PEs who do not receive treatment. Recurrent embolism is the most common cause of death. A 48-year-old woman presented to Tan Tock Seng Hospital, Singapore, on December 16, 2009, with a massive pulmonary thromboembolism. She was admitted to the intensive care unit and treated with supportive measures, ventilatory support, antibiotics and ionotropes. The patient was diagnosed with urosepsis with septicemic shock, disseminated intravascular coagulation, acute renal failure, high anion gap metabolic acidosis, iron-deficiency anemia secondary to menorrhagia, and a uterine mass with high cancer antigen 125, although malignancy was ruled out. Anticoagulation or thrombolysis could not be provided in view of coagulopathy. The patient subsequently underwent inferior vena cava filter insertion on December 31, 2009. The patient showed clinical improvement over the next two weeks with antibiotics and supportive measures. If there is a contraindication to anticoagulation or thrombolysis, massive pulmonary thromboembolism should be treated aggressively with supportive measures. Inferior vena cava filter insertion should be instituted early to prevent recurrent PE, which can be fatal. It may take weeks before the patient displays clinical improvement.
High CA 125; Inferior vena cava filter; Massive pulmonary embolism; No anticoagulation; No thrombolysis
Intravascular lymphoma (IVL) is a rare disease form of malignant lymphoma, and it is characterised by the selective growth of lymphoma cells within the lumina of vessels. Identification of this disease at an early stage is difficult because of non-specific clinical symptoms and neuroradiological findings. Most reported IVL cases are diagnosed at post-mortem following autopsy. We report the case of a patient who presented with status epilepticus (SE) as the initial manifestation of IVL. Despite the administration of anti-convulsant agents and general care the patient's condition deteriorated rapidly after admission, culminating in death due to respiratory failure and heart failure 21 days after the onset of symptoms. Post-mortem examination revealed IVL in the brain and multiple organs. Epileptic seizures often appear during the clinical course of IVL; however, they occur most frequently at advanced stages. Diagnosis of IVL that first presents with SE is of clinical importance because the treatment and prognosis of acute SE arising from IVL are different from those of SE originating from other causes.
Status epilepticus; Intravascular lymphoma; Diffusion-weighted imaging
Idiopathic pulmonary fibrosis (IPF) is a chronic diffuse interstitial disease characterized by a predominant reticular pattern of involvement of the lung parenchyma which can be well documented by High Resolution Computed Tomography (HRCT). While almost half of the patients with IPF may develop pulmonary arterial hypertension, the occurrence of superimposed acute thrombo-embolic disease is rare.
We describe a case of an 87 yrs old female who was found to have IPF complicated by acute pulmonary thrombo-embolism during the clinical and radiological investigation of a rapidly worsening dyspnea. While chest x-ray findings were initially considered consistent with a congestive heart failure, a bed side echocardiography revealed findings suggestive of pulmonary arterial hypertension and right ventricular failure with enlargement of both right cavities and associated valvular regurgitations. An acute thrombo-embolic disease was initially ruled out by a perfusion lung scintigraphy and subsequently confirmed by contrast-enhanced multi-detector CT which showed an embolus at the emergency of the right inter-lobar artery with associated signs of chronic pulmonary hypertension. However, unenhanced scans performed with both conventional and high resolution techniques also depicted a reticular pattern of involvement of lung parenchyma considered suggestive of IPF despite a atypical upper lobe predominance. IPF was later confirmed by further clinical, serological and instrumental follow-up.
Idiopathic pulmonary fibrosis; high resolution computed tomography; pulmonary thrombo-embolism; multidetector row CT angiography
An automated computer analysis of ventilation-perfusion lung scans was used to derive graphical data from lung scans of 11 patients with acute minor pulmonary embolism, free of pre-existing cardiorespiratory disease, and with no evidence of intrapulmonary complication or pleural effusion. In each case the analysis showed the presence of areas of lung, remote from those affected by the pulmonary embolism, that had a pathological disturbance of ventilation-perfusion matching with relative overperfusion. Such a disturbance would cause hypoxaemia. When the extent of the mismatching was calculated in terms of relative blood flow and alveolar ventilation it correlated well with the degree of arterial hypoxaemia. It is proposed that in acute minor pulmonary embolism the development of ventilation-perfusion mismatching in areas of lung unaffected by the embolic event may be an important mechanism of hypoxaemia.
To investigate the clinicopathological features of primary intravascular large B-cell lymphoma of lung.
A case of primary pulmonary intravascular large B-cell lymphoma was analysed in histopathology and immunophenotype.
The patient is a 42-year-old female who had cough for one year. Computed tomography showed ground-glass opacities and small nodules in bilateral lung fields. Histopathology demonstrated accumulation of similar sized neoplastic cells within alveolar capillaries, widening the alveolar septae. The alveolar structure sustained in part of districtions. Immunohistologically, the tumor cells were positive for CD20 and negative for CD3,CK, which were similar to the diffuse large B-cell lymphoma.
Intravascular large B-cell lymphoma is an uncommon type of non-Hodgkin’s lymphoma. Primary pulmonary presentation is even more rare. The diagnosis is based on the histopathology and immunohistochemistry.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2076991810705433.
Intravascular large B-cell lymphoma; Primary; Lung; Clinicopathology
Intravascular lymphomatosis (IL) is a rare and generally fatal disease characterized by proliferation of large lymphoma cells almost exclusively within the lumen of small blood vessels. The skin and central nervous systems are typically affected, but involvement of other organs, such as lung, has been described. Predominant lung involvement without cutaneous and neurologic manifestation is very rare and difficult to diagnose. Originally considered as an endothelial disorder, IL has recently been reclassified as lymphoma. Most of the cases reported are of B cell lineage with a few cases of T cell type. We describe a case of the T-cell type IL manifested clinically as an interstitial lung disease without involvement of skin and central nervous systems. Immunohistochemical studies showed the T-cell nature of the neoplastic cells in open lung biopsy sample.
Acute pulmonary embolism has varied presentations ranging from asymptomatic, incidentally discovered emboli to massive embolism, causing immediate death. Tumor embolism is a rare but unique complication of malignancies. This uncommon catastrophe of a malignant tumor in a young patient, culminating as a pulmonary embolism, is being reported for the first time.
A 19-year-old Asian man presented to the emergency service at our hospital with acute onset dyspnea. His clinical examination led to the suspicion of an acute pulmonary embolism with a lower lumbosacral radiculopathy. A magnetic resonance imaging scan of the pelvis demonstrated a chondrosarcoma arising from the right iliac wing, eroding into the common iliac vein and creeping up the inferior vena cava to lodge in the pulmonary artery, thus producing a saddle embolus.
The importance of exploring for malignancies in the event of an idiopathic pulmonary embolism is highlighted. Early detection of such malignancies can substantially affect the outcome in young patients.
Several case reports have described acute lung injury and respiratory distress following the intravascular injection of oil. Although biochemical and mechanical theories explaining the pathological mechanism of pulmonary oil embolism have been proposed, the phenomenon is not completely understood. This report describes a case of acute respiratory distress and hypoxemia involving a 21-year-old bodybuilder who self-administered an injection of anabolic steroids suspended in oil. The ensuing brief discussion reviews the pathophysiology, diagnosis and treatment of this potentially life-threatening scenario.
A case of acute respiratory distress and hypoxemia following accidental intravenous injection of an oil-steroid solution in a body builder is presented. Chest roentography at the time of presentation showed diffuse bilateral opacities, and computed tomography revealed predominantly peripheral ground-glass opacifications. The patient’s symptoms gradually improved over 48 h and imaging of the chest was unremarkable one week later. The pathophysiology, diagnosis and treatment of this rare but potentially life-threatening complication of intravenous oil injection are discussed.
Pulmonary oil embolism; Steroid-oil injection
Intravascular Large B-cell lymphoma (IVLBCL) is an exceptionally rare form of non-Hodgkin lymphoma (NHL) distinguished by the preferential growth of neoplastic cells within blood vessel lumen. Challenging to detect and deemed disseminated at diagnosis, this condition is characterized by a highly aggressive, inconspicuous course with a high mortality rate. We describe the case of a 48 year-old African-American female presenting with a two month history of low-grade fevers and malaise. Laboratory data was notable for anemia, thrombocytopenia, elevated liver function tests, and hematuria. An extensive work-up for infectious, rheumatologic and malignant causes was negative. Her symptoms progressed and within two weeks, she was admitted for disseminated intravascular coagulation (DIC). Her course was complicated by diffuse pulmonary hemorrhage and ultimately, care was withdrawn. Autopsy identified widespread CD-20 positive intravascular large B-cell lymphoma with significant hepatosplenic involvement, characteristic of the Asian variant IVLBCL. This case uniquely highlights development of the Asian variant IVLBVL in a previously undescribed race. Identified by its intraluminal vascular growth pattern, IVLBCL generally spares lymphatic channels. Diagnosis and differentiation of this condition from other hematological malignancies via skin, visceral and bone marrow biopsy is imperative as anthracycline-containing chemotherapies may significantly improve clinical outcomes. This article outlines the common presentation, natural course, and treatment options of IVLBCL, along with the histopathology, immunohistochemistry, and chromosomal aberrations common to this condition.
intravascular lymphoma; Asian variant; hemophagocytic syndrome; diffuse large B-cell lymphoma; chromosome abnormalities.
Idiopathic acute eosinophilic pneumonia (IAEP), characterized by acute febrile respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, is rarely reported in children. Diagnosis is based on an association of characteristic features including acute respiratory failure with fever, bilateral infiltrates on the chest X-ray, severe hypoxemia and bronchoalveolar lavage fluid >25% eosinophils or a predominant eosinophilic infiltrate in lung biopsies in the absence of any identifiable etiology. We present a 14-month-old girl who was admitted to our pediatric intensive care unit because of acute respiratory distress. She had a fever, dry cough, and progressive dyspnea for 1 day. Chest X-ray showed multifocal consolidations, increased interstitial markings, parenchymal emphysema and pneumothorax. IAEP was confirmed by marked pulmonary infiltrates of eosinophils in the lung biopsy specimen. Most known causes of acute eosinophilic pneumonia, such as exposure to causative drugs, toxins, second-hand smoking and infections were excluded. Her symptoms were resolved quickly after corticosteroid therapy.
Pulmonary eosiophilia; Idiopathic; Respiratory distress; Child
A man in his 30s presented with symptoms of shortness of breath (SOB). He was on clozapine for schizophrenic symptoms. From the initial two presentations it was thought to be and managed as a chest infection, and a perfusion ventilation scan was done to rule out pulmonary embolism. However, with worsening SOB on exertion, he presented for a third time, and was referred and seen in the Department of Medicine on this occasion. The ECG showed evidence of left atrial and ventricular enlargement. The chest x ray showed an increased cardiothoracic ratio. An urgent echocardiogram showed the presence of dilated cardiomyopathy with severe left ventricular systolic function impairment. The patient had a coronary angiogram and other relevant investigations to look for the cause of the dilated cardiomyopathy. These investigations did not reveal any significant abnormality. The cause appeared to be related to the drug clozapine. The patient was treated for heart failure, and clozapine was stopped. He improved and repeat echocardiogram at follow-up showed a definite improvement in the symptoms and the echocardiogram.
A previously reported computer analysis has been used to provide numerical ventilation-perfusion lung scan data, for comparison with tests of airways function and results of arterial blood gas analysis in 11 patients with pulmonary embolism, 18 with asthma, and 37 with chronic obstructive lung disease. In pulmonary embolism an index of underperfusion showed high sensitivity, and an index of ventilation-perfusion mismatching correlated well with severity (hypoxaemia). In asthma an index of underventilation was sensitive and correlated well with severity of airways obstruction. In chronic obstructive lung disease the same index was sensitive but correlated poorly with severity. This was attributed to heterogeneity of the lung disease (airways obstruction plus emphysema) in chronic obstructive lung disease. Ventilation-perfusion mismatching was frequently present in airways disease, and was often of great severity in chronic obstructive lung disease. Discrimination between pulmonary embolism and either type of airways disease was possible by using a combination of underfusion and underventilation indices. The technique offers the prospect of increasing the information derived from lung scans and of automating the reporting of scans.
Antiphospholipid syndrome is a well-defined entity that is characterized by spontaneous abortion, thrombocytopenia, and recurrent arterial and venous thromboses. A partially calcified right atrial thrombus mimicking myxoma with recurrent pulmonary embolism has not been previously reported in a patient who also had systemic lupus erythematosus and secondary antiphospholipid syndrome.
Herein, we describe the case of a 37-year-old woman with systemic lupus erythematosus and secondary antiphospholipid syndrome who was admitted to the hospital with progressive exertional dyspnea. Ventilation-perfusion scanning showed multiple parenchymal defects in the lungs that portended pulmonary embolism. In addition, the scanning revealed normal regional ventilation. Transthoracic and transesophageal echocardiography showed a right atrial mass that was highly suggestive of myxoma, and the patient subsequently underwent surgery. A histologic examination showed an organized, partially calcified thrombus.
Intracardiac thrombus has been rarely reported as a complication of antiphospholipid syndrome. In our patient, the preoperative investigations could not differentiate the partially calcified right atrial thrombus from a myxoma, and the diagnosis was made postoperatively.
Antibodies, anticardiolipin/blood; antiphospholipid syndrome/complications; autoimmune diseases/complications; coronary thrombosis/complications/diagnosis/epidemiology/radiography/surgery; heart atria; heart neoplasms/diagnosis; lupus erythematosus, systemic/complications; myxoma/diagnosis; recurrence; thrombosis/complications/diagnosis/etiology/pathology/prevention & control/surgery
Acute renal failure, disseminated intravascular coagulation (DIC), acute respiratory distress syndrome (ARDS), hypoglycemia, coma, or epileptic seizures are manifestations of severe Plasmodium falciparum malaria. On the other hand, Plasmodium vivax malaria seldom results in pulmonary damage, and pulmonary complications are exceedingly rare. We report the case of a 42-year-old male living in a malaria-endemic area who presented with ARDS and was diagnosed as having Plasmodium vivax malaria. A diagnosis of Plasmodium vivax malaria was established by a positive Plasmodium LDH immunochromatographic assay while a negative PfHRP2 based assay ruled out P. falciparum malaria. After specific anti-plasmodial therapy and intensive supportive care, the patient recovered and was discharged from hospital. The use of NIPPV in vivax-malaria related ARDS was associated with a good outcome.
Acute respiratory distress syndrome; Plasmodium vivax; PfHRP-2; NIV
Pulmonary embolism is a common condition. Some patients subsequently develop chronic thromboembolic pulmonary hypertension (CTEPH). Many care gaps exist in the diagnosis and management of CTEPH patients including lack of awareness, incomplete diagnostic assessment, and inconsistent use of surgical and medical therapies.
A representative interdisciplinary panel of medical experts undertook a formal clinical practice guideline development process. A total of 20 key clinical issues were defined according to the patient population, intervention, comparator, outcome (PICO) approach. The panel performed an evidence-based, systematic, literature review, assessed and graded the relevant evidence, and made 26 recommendations.
Asymptomatic patients postpulmonary embolism should not be screened for CTEPH. In patients with pulmonary hypertension, the possibility of CTEPH should be routinely evaluated with initial ventilation/ perfusion lung scanning, not computed tomography angiography. Pulmonary endarterectomy surgery is the treatment of choice in patients with surgically accessible CTEPH, and may also be effective in CTEPH patients with disease in more ‘distal’ pulmonary arteries. The anatomical extent of CTEPH for surgical pulmonary endarterectomy is best assessed by contrast pulmonary angiography, although positive computed tomography angiography may be acceptable. Novel medications indicated for the treatment of pulmonary hypertension may be effective for selected CTEPH patients.
The present guideline requires formal dissemination to relevant target user groups, the development of tools for implementation into routine clinical practice and formal evaluation of the impact of the guideline on the quality of care of CTEPH patients. Moreover, the guideline will be updated periodically to reflect new evidence or clinical approaches.
Chronic thromboembolic pulmonary hypertension; Clinical practice guideline; Pulmonary endarterectomy; Pulmonary hypertension; Thromboembolism
The conventional method of lung scanning detects defects of pulmonary artery perfusion. False positive results occur because regions of hypoventilation, such as are present in obstructive airways disease, also cause defects of perfusion. The converse is not true, however, as defects of perfusion continue to be ventilated. Thus in pulmonary embolism ventilation-perfusion discrepancy (normal ventilation and impaired perfusion) occurs.
We have assessed the clinical value of this discrepancy. Out of 18 patients with ventilation-perfusion discrepancy 14 had a final diagnosis of pulmonary emboli, whereas in none of the 34 patients without the discrepancy was this final diagnosis made. We conclude that combined ventilation-perfusion lung scanning distinguishes pulmonary emboli from other lung conditions such as asthma and bronchitis which cause impaired pulmonary perfusion. The false positive rate was only 4% overall and 7·7% in patients with perfusion defects.
Leukemic pulmonary infiltration, as an initial presentation of acute leukemia, is rare and poses a therapeutic dilemma. Leukemic infiltrate of the lung may be unrecognized, as patients can present with cough, fever and localized roentgenographic infiltrate, all suggestive of bacterial pneumonia. Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML) with distinct clinicopathologic features, such as: younger age of patients, shorter duration of symptoms before diagnosis, pulmonary infiltration with atypical promyelocytes and bleeding tendency due to disseminated intravascular coagulation (DIC). APL can become rapidly fatal if not treated early in its course. We report a case of APL with diffuse pulmonary infiltration and abnormal complete blood count. He was initially diagnosed and treated as an outpatient for community-acquired pneumonia. The patient returned with worsening pulmonary infiltrate, abnormal peripheral smear and respiratory failure, resulting in death within three months of his initial presentation. As evidenced by this case, acute leukemia should be considered in the differential diagnosis for pulmonary infiltrate and abnormal hematological findings.
A 40 year old woman presented with a short history of acute onset of breathlessness to the ER of our hospital and after initial evaluation for acute pulmonary embolism which was ruled out after carrying out the appropriate investigations, she was diagnosed to be afflicted with syringomyelia based on her neurological symptoms and clinical findings, which was confirmed by doing an MRI scan, which was her basic diagnosis that was complicated by acute hypercapnic respiratory failure. This case is being reported to highlight syringomyelia as an unusual cause of acute respiratory failure, which manifested clinically in this patient as its first presentation and the underlying neurological diagnosis has been found to be present in very few reported cases (less than 0.01% of case reports) in the available literature as the basic disease in the absence of its classical presenting features. Problems associated with acute respiratory failure in the setting of syringomyelia are discussed.
Respiratory failure; syringomyelia; syrinx; hypercapnia
Seventy-one patients with suspected pulmonary embolism had both pulmonary scans and angiograms. Clinically it was impossible to predict the absence or presence of subsequent angiographic evidence of pulmonary embolism in the individual patient. In 24 patients the scans showed defects that were believed to indicate a high probability of pulmonary embolism—namely, perfusion defects that corresponded to specific anatomical segments of the lung together with normal chest radiographs or radiographs suggestive of pulmonary embolism. Specific angiographic evidence of pulmonary embolism was found in 18 (75%) of these patients. Diffuse, patchy, and non-segmental perfusion defects were seen in 28 patients. Only seven (25%) of this group had angiographic abnormalities specific for embolism. In 12 patients with normal lung scans none showed subsequent evidence of embolism by angiography. It is concluded firstly that lung scanning is a sensitive screening procedure for pulmonary embolism and, secondly, that segmental perfusion defects are highly suggestive of pulmonary embolism.
It has been suggested that obliterative vasculitis in the lung might mimic pulmonary embolism on a ventilationperfusion scan. The combined scans of six patients with breast cancer who had undergone radiotherapy to the chest wall, which can induce pulmonary vasculitis, were therefore examined. Eleven of the 12 scans showed perfusion defects with ventilation-perfusion mismatch on the irradiated side. Special care is needed in interpreting the lung scans of patients who have received an appreciable tissue dose of radiation to the lungs; mismatch need not indicate pulmonary embolism.
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by pulmonary hypertension caused by nonresolving thromboemboli of the pulmonary artery. However, up to 40% of CTEPH patients have had no clinically apparent acute pulmonary embolic episodes. In addition, both a female predominance and an association of HLA with CTEPH unrelated to deep vein thrombosis are observed in Japan, thus suggesting this condition to be a type of pulmonary vasculopathy. The key for making an accurate diagnosis is to consider CTEPH in any patients with dyspnea on exertion. Perfusion scans provide an excellent noninvasive tool for distinguishing between pulmonary arterial hypertension (PAH) and CTEPH, while CT angiography is useful for differentiating arteritis, tumor, and a congenital anomaly of the pulmonary artery from CTEPH. Pulmonary angiography (PAG) is still considered the gold standard for evaluating CTEPH. No subpleural perfusion in any segments by PAG with high pulmonary vascular resistance is might be related to small vessel disease, thus resulting in a poor outcome after surgery. Recent data suggest a potential therapeutic benefit of antiproliferative therapy for cells isolated from endarterectomized tissue.
chronic thromboembolic pulmonary hypertension; perfusion scan; pulmonary angiography; HLA; pulmonary vascular remodeling