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Carcinomas of the lung that infiltrate the blood vessels close to the heart (left atrium, pulmonary artery and aorta) without spreading to mediastinal lymph nodes or developing distant metastases are rare overall. Such situations are often classified as primarily inoperable by interdisciplinary tumour boards. This is only the case if, for technical reasons, an experienced thoracic surgeon does not feel able to perform a resection with a surrounding margin of healthy tissue. The surgical strategy to be employed must be chosen individually depending on the infiltrated structure. Complete tumour staging should always be carried out. This also helps in deciding whether neoadjuvant chemotherapy should be given before resection. A heart-lung machine must always be used if larger defects occur due to the resection of blood vessels close to the heart. Using a heart-lung machine in the case of tumour resection does not lead to problems of tumour cell dissemination. Nevertheless, the duration of use of the heart-lung machine should be kept to a minimum, also because of the anticoagulation required. The cardiac defects can be closed securely with the bovine patching materials that are now available. Postoperative morbidity and mortality are low after such resections. Curative resection of blood vessels close to the heart infiltrated by carcinomas of the lung can lead to 5-year survival rates of up to 50 %., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: In every pneumonectomy due to a malignant or benign pulmonary disease the pulmonary artery must be centrally ligated and dissected. If a thoracotomy is undertaken the vessel is usually doubly ligated with a non-absorbable suture and then dissected. Alternatively a vessel stapler can be used. In a thorascopic procedure only the stapler can be used. In the search for a cheaper alternative we investigated whether a bipolar instrument as employed in thorascopy could be used and reused. The aim of this study was to investigate the static pressure performance of the closed vessel in an ex vivo non-perfused vessel model., Material and Methods: From freshly slaughtered pigs the heart-lung block was removed. The central pulmonary artery was exposed. For sealing the vessel we used the bipolar sealing system MARSEAL®, Brothers Martin company (Tuttlingen, Germany), consisting of an HF generator and a MARSEAL slim instrument. Two groups were formed: group A (n = 49) Stromart SealSafe®, step G5 (automatic performance regulation, duration of use depending on impedance) and group B (n = 58) bipolar Stromart "Macrocoag" (power: 120 W, constant duration of use: 15 s). At the end of the pulmonary artery a pressure probe was implanted to digitally measure the pressure at the vessel sealing suture. After sealing the end of the artery, the vessel was slowly filled via an external source in order to determine the burst pressure. The burst pressure was reached as soon as the vessel suture started to leak. The average values of the 2 groups were then compared (t test for independent variables, significance set at p < 0.05)., Results: All examined vessels (n = 107) had the same vessel diameter of 15 ± 3 mm. The average value of the burst pressure in group A was 139.3 ± 62.17 mmHg, that in group B 124.8 ± 38.46 mmHg. The ranges of burst pressures were in group A 51-297.0 mmHg und in group B 60.75-244.5 mmHg. There was no significant difference between group A and group B (p = 0.071)., Conclusions: Satisfactory burst pressures in the central pulmonary artery can also be achieved with the bipolar sealing instrument MARSEAL® and the impedance-controlled Stromart SealSafe®. However, because of the large scattering of the measured values, further studies are required., (Georg Thieme Verlag KG Stuttgart · New York.)

Sleeve resections of the lungs have affected the oncologic radicality, parenchyma and lung function-saving resections and extended the indications for operations in thoracic surgery. Whenever lung amputations can be avoided by bronchoplastic and/or angioplastic procedures with the same radicality, sleeve resection should be performed. In centrally located distinct malignomas, intraluminal tumor growth (T3) infiltrations of peribronchial or extrabronchial areas, the lobular ostia and the pulmonary artery (T2/T3) as well as lymph node involvement (N1/N2), these procedures give a better qualitative survival and lower morbidity and mortality rates. Broncoscope-guided localization of a double lumen tube and routine anesthesia monitoring are mandatory. Before performing sleeve resections a complete lymph node dissection should be done without denuding the area of the anastomosis and sparing the bronchial arteries. Preoperative endoscopic biopsies, knowledge of the topography and mobilization of the vascular and bronchial tree, subtile operation techniques, perioperative and postoperative videobronchoscopic guidance as well as intraoperative frozen sections and a tension-free and smooth anastomosis, avoid postoperative complications. Depending on the blood supply of the bronchial tree a vascularized flap is indicated. Operability can therefore be achieved in elderly patients with limited pulmonary function, particularly those under adjuvant or neoadjuvant therapy who are no longer suitable for pneumonectomy.

Lung cancer is localized in the upper lobes in more than half of the cases. The risk of tumor infiltration of centrally located structures, such as bronchi and vessels are enhanced due to the anatomic topography. Pneumonectomy competes with sleeve resection for the surgical resection of centrally located tumors. The present review deals with the question if pneumonectomy should be considered as an alternative to sleeve resection for the treatment of lung cancer. Primary pneumonectomy does not provide any advantage even in advanced nodal disease. Extended lymph node dissection is not a contraindication for sleeve resections. Local recurrence rate is lower after sleeve resections despite the same radicality for both surgical treatment options. Mortality and morbidity rates are significantly lower for sleeve resections. Sleeve resections are associated with prolonged survival and better quality of life even in elderly patients.

History and Admission Findings: A 43-year-old woman presented with persistent dry cough and exertional dyspnoea for about 6 months. An outpatient CT scan of the chest led to the diagnosis of pulmonary thromboembolism., Investigations: The echocardiography showed signs of right ventricular load. Phlebothrombosis, thrombophilia or an active malignant disease were excluded., Treatment and Course: An anticoagulant therapy was started in the hemodynamically stable patient. She was discharged in good condition. Four weeks later the patient was readmitted with worsening of symptoms. The CT scan showed progression of the findings in spite of sufficient anticoagulant therapy. Echocardiography revealed an increase of the right ventricular load. A systemic thrombolysis with 100 mg alteplase followed, but without any change. Subsequently, the suspicion of an intraluminal growing tumorous mass was confirmed. A tumor-endarterectomy with reconstruction of the left pulmonary artery together with a pericardial patch and an extensive pneumonectomy on the right were performed. Histopathologically and immunohistochemically a pulmonary artery sarcoma with the differentiation of a leiomyosarcoma was diagnosed., Conclusion: The primary pulmonary artery sarcoma is a rare disease and pulmonary thromboembolism must be considered for differential diagnosis. In the CT scan a low-attenuation filling defect occupying the entire luminal diameter of the pulmonary artery, expansion of the involved arteries or extraluminal tumor extension can lead to a suspected diagnosis. Furthermore in case of a progression of the findings under sufficient anticoagulant therapy a primary pulmonary sarcoma has to be considered as differential diagnosis. Complete surgical resection offers the only chance of cure., (© Georg Thieme Verlag KG Stuttgart · New York.)

During embryological development the heart develops from a simple tube into a complex fully developed heart with four chambers. Hence all congenital heart defects develop before the ninth week of gestation. Currently a steadily increasing number of genetic mutations have been found to be responsible for congenital heart defects. Nevertheless, up to now it has been impossible to diagnose a heart defect just on the basis of molecular pathology. Despite the current excellent prenatal and postnatal ultrasound diagnostics, the post-mortem examination is still the gold standard for the diagnosis of complex heart malformations. However, this requires knowledge of the pathomorphology of the heart malformation in question. Therefore, characteristic and distinguishing features of septal defects including atrioventricular septal defects are presented, especially as the latter are part of complex heart defects, such as conotruncal heart malformations.

In pulmonary hypertension there is a discrepancy between the dramatic but unspecific clinical presentation and the remodeling of mostly only limited segments of the vascular compartment of pulmonary parenchyma. Clinical diagnosis relies for the most part on invasive procedures, such as right heart catheterization. Therefore, morphology can provide a reliable etiopathogenetic classification only in close cooperation with the clinical partner disciplines involved. Moreover, the histopathological approach requires intimate knowledge of the vascular anatomy of the lungs and assessment of the parenchyma to be able to diagnose pulmonary hypertension and differentiate between the various types.

This article provides a brief overview on the history of pulmonary hypertension, starting with the first descriptions of the accompanying pulmonary vascular lesions by Ernst von Romberg and Victor Eisenmenger at the end of the 19th century. Many of the histopathological changes in the pulmonary vasculature found in the various forms of pulmonary hypertension had already been described in the first half of the 20th century. However, only through the pioneering work by Forssmann and Cournand during the middle of the 20th century was it finally possible to catheterise the right ventricle and the pulmonary arteries. After this it became feasible to study the clinical spectrum of the various forms of pulmonary hypertension as well as the effects of therapeutic interventions. Early treatment attempts with vasodilators, however, were not successful. Intravenous prostacyclin, used to treat some forms of pulmonary hypertension since 1980, became the first effective treatment. Since that time, our understanding of the pathogenesis of pulmonary hypertension has increased substantially, as has the number of effective therapies, at least for some forms of pulmonary hypertension. Many aspects of the disease, however, remain poorly understood and a cure is still not achievable for the majority of the affected patients., (Copyright Georg Thieme Verlag KG Stuttgart . New York.)

Rheumatic diseases or collagen vascular diseases represent a heterogeneous group of immunologically mediated inflammatory disorders. The respiratory system is often affected,the causes being manifold: infection, medication toxicity and specific manifestations of immunological processes due to the underlying disease. The lung can be involved in all its components. Due to their extremely broad differential diagnosis, pulmonary vasculitic syndromes still constitute a major challenge for the pathologist. Pulmonary involvement is frequent in primary systemic vasculitis (PSV) associated with anti-neutrophil-cytoplasmic antibodies (ANCA); other PSV only rarely affect the lungs. Histomorphologically, small vessel vasculitis with neutrophil alveolitis and diffuse alveolar hemorrhage, as well as extravascular intraparenchymal or peribronchial granulomas, can point to PSV. A single biopsy is often insufficient to identify all diagnostic criteria. Therefore, the selection of suitable biopsy material and correlation with clinical, serological and radiological parameters is indispensable. Almost all forms of interstitial lung disease may be present in collagen vascular disease; however, several parallel morphological types, rather than one in isolation, are frequently found.

Pulmonary arterial hypertension is a life-threatening, vasculoproliferative disease of the lung, which is characterized by vasoconstriction and remodeling of small pulmonary arteries. Drugs for the treatment of PAH mainly address the increased vascular tone. Substances like prostacyclin, endothelin-receptor-antagonists and phosphodiesterase-5-inhibitors have been approved for the treatment of PAH and represent the current therapeutic options. The development of a causal treatment aiming a normalization of the vessel wall structure is the current focus of research. The key events in disease progression are represented by increased proliferation, migration and a resistance to apoptosis of pulmonary vascular cells. Therefore, new non-vasoactive drugs are investigated in relevant preclinical animal models of pulmonary arterial hypertension. Some of these substances, like tyrosine kinase inhibitors, elastase inhibitors and phosphodiesterase-1-inhibitors, could not only attenuate (anti-remodeling) but reverse (reverse-remodeling) the disease. Additionally, new vasodilators, like soluble guanylate cyclase stimulators and activators, addressing well-known and new signaling pathways are currently under investigation. Taken together, with increasing insight into the pathology of PAH, several novel drug targets and treatments have emerged which may improve the management of patients and which efficacy is currently addressed in preclinical studies and clinical trials., (Georg Thieme Verlag KG Stuttgart, New York.)

With technical improvements in gradient hardware and the implementation of innovative k-space sampling techniques, such as parallel imaging, the feasibility of pulmonary perfusion MRI could be demonstrated in several studies. Dynamic contrast-enhanced 3D gradient echo sequences as used for time-resolved MR angiography have been established as the preferred pulse sequences for lung perfusion MRI. With these techniques perfusion of the entire lung can be visualized with a sufficiently high temporal and spatial resolution. In several trials in patients with acute pulmonary embolism, pulmonary hypertension and airway diseases, the clinical benefit and good correlation with perfusion scintigraphy have been demonstrated. The following review article describes the technical prerequisites, current post-processing techniques and the clinical indications for MR pulmonary perfusion imaging using MRI.

Purpose: Development of pulmonary arterial hypertension (PH) is a common problem in the course of patients suffering from cystic fibrosis (CF). This study was performed to evaluate MRI based flow measurements (MR(venc); Velocity ENCoding) to detect signs of an evolving PH in patients suffering from CF., Materials and Methods: 48 patients (median age: 16 years, range: 10 - 40 years, 25 female) suffering from CF of different severity (mean FEV (1): 74% +/- 23, mean Shwachman-score: 63 +/- 10) were examined using MRI based flow measurements of the main pulmonary artery (MPA). Phase-contrast flash sequences (TR: 9.6 ms, TE: 2.5 ms, bandwidth: 1395 Hertz/Pixel) were utilized. Results were compared to an age- and sex-matched group of 48 healthy subjects. Analyzed flow data where: heart frequency (HF), cardiac output (HZV), acceleration time (AT), proportional acceleration time related to heart rate (ATr), mean systolic blood velocity (MFG), peak velocity (Peak), maximum fow (Fluss(max)), mean flow (Fluss(mitt)) and distensibility (Dist)., Results: The comparison of means revealed significant differences only for MFG, Fluss(max) and Dist, but overlap was marked. However, using a scatter-plot of AT versus MFG, it was possible to identify five CF-patients demonstrating definite signs of PH: AT = 81 ms +/- 14, MFG = 46 +/- 11 cm/s, Dist = 41% +/- 7. These CF-patients where the most severely affected in the investigated group, two of them were listed for complete heart and lung transplantation. The comparison of this subgroup and the remaining CF-patients revealed a highly significant difference for the AT (p = 0.000001) without overlap., Conclusion: Screening of CF-patients for the development of PH using MRvenc of the MPA is not possible. In later stages of disease, the quantification of AT, MFG and Dist in the MPA may be useful for the detection, follow-up and control of therapy of PH. MR(venc) of the MPA completes the MRI-based follow-up of lung parenchyma damage in patients suffering from CF.

Pulmonary arterial hypertension (PAH) is a devastating disease that is characterized by a high mortality. The pathogenesis of PAH is multifactorial. In addition to hereditary factors (e. g., BMPR2 mutations), numerous environmental factors may trigger the onset and progression of the disease. An imbalance between vasoconstrictive and vasodilative factors leads to vasoconstriction in the pulmonary circuit, resulting in an increase of pulmonary vascular resistance and pulmonary artery pressure. Alterations of several signaling pathways (i. e.; endothelin, nitric oxide and prostacyclin pathways) contribute to an increase of pulmonary vascular tone, and these pathways represent the targets of the current therapeutic interventions. However, PAH is increasingly recognized as a chronic proliferative disease particularly of the small pulmonary arteries, that is primarily characterized by morphological changes of the vascular wall ("vascular remodeling"). These changes are particularly induced by peptide growth factors such as platelet-derived growth factor (PDGF) that elicit their signals via activation of membrane-bound receptor tyrosine kinases (RTK). Accordingly, there is both experimental and clinical evidence for a therapeutic efficacy of tyrosine kinase inhibitors (TKI), which provide the basis for "reverse remodeling" strategies and indeed represent a promising novel approach for the treatment of PAH. Epidermal growth factor (EGF), soluble guanylate cyclase (sGC), and phosphodiesterase type 1 (PDE1) may represent additional future target molecules. PAH leads to progressive right heart failure which determines the outcome of PAH patients. The pathomechanisms of right heart failure should therefore also be considered for the development of novel therapeutic concepts.

Following an international consensus conference held in Chapel Hill, North Carolina, USA, vasculitides have been divided based on the size of the vessel involved in the inflammatory process. Thus, the terms large, medium and small vessel vasculitis emerged. Wegener's granulomatosis, microscopic polyangitis and Churg-Strauss syndrome are described as small vessel vasculitides. Pulmonary involvement is a characteristic feature of both Wegener's granulomatosis and Churg-Strauss syndrome, less so in microscopic polyangitis. A pathognomonic feature is alveolar haemorrhage, which encompasses the trias: 1) bilateral acinar pulmonary infiltrates reminiscent of pulmonary edema, 2) anemia and 3) haemoptysis, which however may be absent. Bronchoalveolar lavage typically returns lavage fluid that remains hemorrhagic even after sequential sampling. The discovery that antineutrophil cytoplasmic antibodies (ANCA) are strongly associated with vasculitic disorders and their subsequent characterisation has led to improved understanding of the pathogenic mechanisms underlying vasculitis. This paper will review clinical aspects of pulmonary involvement in the systemic vasculitides.

A case of two non-atherosclerotic aneurysms localised in the ascending aorta and in the pulmonary trunk is presented. Histopathologically, a severe granulomatous inflammation affecting the whole aneurysms wall was documented. To the best of our knowledge it is the second ever documented case of simultaneous occurrence of aneurysms in the aorta and the pulmonary artery.

There are two important options in the surgical treatment of chronic pulmonary hypertension, namely thrombendarterectomy or, if there are no other alternatives, transplantation, which nowadays is almost always double-lung transplantation. Pulmonary thrombendarterectomy is indicated in patients who have a mean pulmonary artery pressure (PAPm) of 40 mm Hg or more, have proximal changes (types I - II) and are in NYHA functional class III - IV. An initial estimate of whether surgical intervention is reasonable can be obtained with spiral computed tomography and pulmonary angiography. Immediate postoperative death rate averages 10 %. In well selected patients operation improves hemodynamics, quality of life and long-term prognosis. Transplantation is indicated in the end-stage. But this therapeutic option is limited by the scarcity of lungs suitable as transplants.

In recent years, pulmonary hypertension got into the focus of research due to the development of efficacious medications and the discovery of important pathologic mechanisms of disease. Currently, prostanoids, endothelin receptor antagonists and phosphodiesterase 5 inhibitors are the most important substance groups used for treatment. Substances that are emerging in tumor therapy (tyrosine kinase inhibitors, epidermal growth factor [EGF] und platelet-derived growth factor [PDGF] receptor blockers), vasoactive intestinal peptide (VIP), rho-kinase inhibitors and targeted drugs for endothelial dysfunction will be evaluated as future drugs for pulmonary hypertension. Improving early diagnosis of pulmonary hypertension will be an important task in the future. Both the development of diagnostic methods with increased sensitivity and specificity and a broad awareness program will be necessary to achieve this goal.

Lung perfusion is a crucial prerequisite for effective gas exchange. Quantification of pulmonary perfusion is important for diagnostic considerations and treatment planning in various diseases of the lungs. Besides disorders of pulmonary vessels such as acute pulmonary embolism and pulmonary hypertension, these also include diseases of the respiratory tract and lung tissue as well as pulmonary tumors. This contribution presents the possibilities and technical requirements of MRI for diagnostic work-up of pulmonary perfusion.

Segmental mediolytic arteriopathy (SMA) is defined as non-inflammatory arteriopathy with mediolysis due to segmental loss of media and consecutive formation of vascular gaps. Up to now, less than 40 cases of visceral and cerebral SMA and, to our knowledge, only one case of pulmonary SMA have been reported. We present the history of a 21 year old female patient, admitted to hospital with hemoptysis, but without other symptoms. Apart from two lesions in the sixth and tenth pulmonary segment, documented by CT and interpreted as colliquations, there were no other clinical and laboratory findings. Repeated bronchoscopy supplied no further information. Histomorphology of the resected lesion revealed SMA without evidence of vasculitis. Wegener's disease could be excluded. The aetiology of the disease is still unknown. Acute vasospasm (due to inappropriate reactions to catecholamine or endothelial dysfunction), as well as SMA as a precursor or subtype of fibromuscular dysplasia, are two theories still under discussion.

Pulmonary arterial hypertension (PAH) is a term which has recently been redefined and includes idiopathic pulmonary arterial hypertension, familial pulmonary arterial hypertension PAH related to specific pathological conditions (e.g. connective tissue diseases), as well as PAH caused by veno-occlusive disease or capillary hemangiomatosis. The clinical manifestation seems to be related to a peculiar pathological anatomy involving small, muscular pulmonary arteries, capillaries and veins. In addition to common hypertrophy of the tunica media, other vascular compartments may also be affected by intimal thickening or adventitial fibrosis. Moreover, complex lesions, such as so called plexiform lesions and arteritis can be present in certain forms of the disease. While the recent identification of responsible gene mutations in subgroups of patients have shed some light on disease evolution, therapeutic strategies must currently rely on vasodilative and antimitogenic drugs acting on the intimal and medial level of the affected pulmonary vessels. The clinical outcome of patients suffering from PAH remains poor, underlining our need for a better comprehension of disease pathophysiology, and thus for the characterization of specific histomorphological patterns.

In recent years microcomputed tomography (microCT) has become more and more important in basic research. Now commercial microCT scanners are available. Thus, it is very likely that this new, accurate and promising method for three-dimensional and non-destructive quantitative evaluation of intact tissues including vessels will be applied more frequently. The review provides a survey of the basic technology of microCT and its current use for high resolution three-dimensional morphometric and functional analysis within the cardio-pulmonary vascular system.

Purpose: To evaluate time-resolved, multiphasic 3D MR angiography (MRA) for the non-invasive assessment of the pulmonary vascular system in children., Materials and Methods: 10 children aged 6 to 15 years (mean age 10 years) ware examined on a 1.5 T whole body MR system with time-resolved, multiphasic 3D MRA after injection of 0.2 mmol/kg body weight of Gd-DTPA. With the use of an ultrafast gradient echo pulse sequence with asymmetric k-space filling and very short echo and repetition times, a nominal spatial resolution of 1.4 x 1.4 x 2.0 mm3 could be achieved with a scan time of 5.6 and 6.2 seconds for a single 3D data set. Two radiologists, who were blinded to the clinical diagnosis, analyzed the image data in consensus. The image analysis included the assessment of the image contrast and artifacts as well as a quantitative analysis of the signal-to-noise (SNR) and contrast-to-noise ratios (CNR) for central and peripheral lung vessel segments., Results: The children tolerated all examinations without any side effects. The referral diagnosis, which was based on echocardiography, catheter angiography and surgery, was confirmed by MRA in all cases. The image contrast was rated at least satisfactory in all but one case (19 of 20) and no artifacts were observed. The quantitative analysis of the SNR and CNR in the pulmonary arteries and veins confirmed the reader analysis of a high and uniform contrast throughout the entire pulmonary circulation., Conclusion: Time-resolved multiphasic 3D MRA allows a non-invasive diagnostic evaluation of the pulmonary circulation in children. In view of the excellent image quality, MRA may replace conventional diagnostic catheter angiography in the near future.

An 87-year-old woman, who was largely immobilized in a wheelchair, suffered rib fractures and an unstable fracture of the pelvic ring in a fall. Approximately 2 1/2 hours later she developed marked clinical symptoms of pulmonary thromboembolism and died 5 hours after the accident. The question to be discussed with regard to the causality was the unusually short interval of only 2 1/2 to 5 hours between the accident and the clinical signs of embolism leading to her death. Current literature gives only rough outlines stating that thromboembolism can occur as early as the first day after the trauma. An alternative theory explaining the pulmonary thromboembolism may be the breaking off of a pre-existing thrombus due to manipulation during transport or diagnostic measures in connection with the unstable fracture of the pelvic ring.

Embolisation of a foreign body is a rare cause of pulmonary embolism. We report on a 67 year old female suffering from colorectal cancer. To perform adjuvant chemotherapy a port system was inserted into the right subclavian vein. Routine chest radiography revealed a rupture of the venous port-system, which was embolised into the right pulmonary artery without clinical symptoms. The foreign body was removed without complications using a nitinol goose neck snare via a transfemoral approach and retrieved percutaneously. The reported procedure is safe, decreases operation and hospitalisation time and should therefore be performed in preference to surgical management whenever possible.

Leiomyosarcomas are only rarely found in routine cardiac surgery. For the patient they hold, however, a very high-risk potential as only an early diagnosis, and immediate surgical therapy leads to long-term survival. Awareness, timely diagnosis, and immediate radical surgical therapy is imperative. This report describes the case of a 21-year old male with a strong suspicion of leiomyosarcoma of the pulmonary artery. Due to his clinical symptoms, the patient was transferred to operation immediately after completion of all diagnostic measurements. Postoperatively the diagnosis of a leiomyosarcoma proved to be false; instead a rare fibro-histologic tumor was found. Even in the case of a suspected leiomyosarcoma, rapid and radical surgical therapy is imperative due to the poor prognosis of leiomyosarcoma.

Background and Objectives: Pulmonary artery sarcoma is a rare neoplasm and possibly unnoticed cause of pulmonary hypertension. The presentation is one of central pulmonary artery obstruction and progressive right-heart failure. In most cases, the diagnosis of malignancy is confirmed post mortem. We report the outcome of eight patients with primary pulmonary artery sarcomas., Methods: Eight patients (four female, four male, mean age 48,2 years, preop. NYHA functional class III/IV: n = 5/3) were referred for further evaluation of pulmonary hypertension. Malignancy was suspected in six of these patients by means of computed tomography (CT) and magnetic resonance tomography (MRT). In two patients diagnosis was established during pulmonary thromboendarterectomy based on histological examination of frozen sections. Operative procedures consisted of gross tumor resection with prosthetic replacement (n = 3) or reconstruction (n = 5) of central parts of the pulmonary vessels. Additional pneumonectomy was necessary in two patients, resection of metastases in one patient. Seven patients received adjuvant radio- and/or chemotherapy., Results: There were no postoperative deaths. 3 months after surgery, all patients demonstrated improvement in hemodynamics and exercise tolerance. Four patients died 7, 9, 18 and 19 months after surgery, respectively. Two patients are alive 3 and 39 months after primary surgery with evidence of pulmonary metastases. Two patients are alive in complete remission 25 and 65 months postoperatively., Conclusions: In patients with primary pulmonary artery sarcoma, emphasis must be placed on early identification which can be achieved by CT and MRT. Radical surgical resection currently offers the best chance for survival. Adjuvant therapy might bring additional benefit.

An aorto-pulmonary window (aorto-pulmonary septal defect) is considered to be a rare congenital cardiac malformation. We report the case of a patient with an unrepaired congenital aorto-pulmonary window 1.4 cm in size which is the largest aorto-pulmonary septal defect yet reported. This patient died at the age of 58 not from the congenital cardiac malformation but from the after-effects of a pulmonary adenocarcinoma diagnosed 2 years previously. Interestingly, the long standing, persistent pulmonary hypertension correlated well with the varying pathomorphological changes seen in the pulmonary vessels. In addition to the complex morphological signs due to the pulmonary hypertension such as the extensive aneurysms found in the left and right main pulmonary arteries (first report of this size), pulmonary vessels with relatively few pathomorphological changes were also found. Hence this first report of such a long survival of a patient with a comparatively large aorto-pulmonary septal defect demonstrates a surprising variety of pathomorphological alterations seen in the pulmonary arteries in the face of excessive pulmonary hypertension. These alterations could be the result of peripheral aneurysmatic formations and thrombembolic stenoses of the lobular or segmental arteries and might provide an explanation for the long survival of this particular congenital cardiac malformation. All these varying findings in the pulmonary arteries emphasise the fact that the pathomorphological findings in lung biopsies might not be representative of the whole lung.

A 69-year-old male presented with symptoms of fulminant lung embolism and, despite immediate therapy with plasminogen activator, died of acute right heart failure. At autopsy multiple tumor cell emboli were detected in small pulmonary vessels in addition to widespread liver metastases from an urothelial carcinoma. - In a 23-year-old female a malignant gastric ulcer and multiple liver metastases were diagnosed at initial presentation. She too died from pulmonary hypertension due to a series of lung embolisms which occurred despite heparin therapy. At autopsy, many small pulmonary arteries were filled with adenocarcinoma cells; the primary gastric tumor and liver metastases were confirmed. These cases demonstrate that the shedding of tumor cells from hepatic metastases can obstruct the pulmonary vessels and lead to acute cor pulmonale. Tumor cell emboli should be considered in the differential diagnosis of acute pulmonary hypertension, especially in patients with a known tumor. They may, however, also represent the first clinical signs of previously unrecognized malignancy.

We present a rare case of pulmonary embolism in a patient with disseminated hypernephroma. Necropsy showed a large tumor thrombus in the right ventricle. Modern chemotherapy treatments, whilst improving patient's life expectancy to a certain degree don't hinder the spread of metastases which appear then in areas not previously known of. Providing there are no obvious signs of peripheral thrombi and to exclude intracardiac metastases, we recommend echocardiography for all cancer patients with pulmonary emboli.

A 31-year-old pregnant woman (multipara), who was near the expected date of birth, was found dead in the joint flat by her partner. She was lying beside the bed and showed burns on the front of the body caused postmortem by contact with a radiator. Autopsy revealed a rupture of the fetal membranes, the inner layers of the uterus and a large vein. After centrifugation, the supernatant of the blood from the right half of the heart was turbid and contained small corpuscular amniotic fluid components. The diagnosis of amniotic fluid embolism could be confirmed histologically. In the pulmonary vessels of the mother meconium, lanugo hairs and epidermic scales could be demonstrated; in addition the fetal horny lamellae were confirmed immunohistochemically (using monoclonal antibody against cytokeratin).

The employment of the heart-lung machine to assisting the circulatory system for the resection of extracardial tumors is increasing because of their location, growth and infiltration. We report on three patients in whom the resection of the tumor was only possible with extracorporeal circulation (ECC). In the first patient sarcoma of the pulmonary artery and valve was resected under ECC. Artery and valve were replaced with a cryo-preserved valved bifurcation homograft. In the other patients ECC was used on the basis of tumorinfiltration of the inferior vena cava and tumor embolus of the pulmonary artery in a case of primary renal cell carcinoma, and tumor infiltration of the superior vena cava and right atrium in a case of follicular thyroid carcinoma. All patients had a good postoperative course and excellent long term survival.

Radiological cross-sectional imaging modalities, particularly computed tomography (CT) have become the mainstays for diagnosing lung disease in recent years. These enable morphological visualization of pathological processes with the greatest possible spatial resolution. Modern technical developments and complementary strategies have led to new applications and new functional assessments which need to be reviewed together with state-of-the-art techniques in nuclear imaging. The diagnosis of pulmonary embolism using spiral CT angiography and magnetic resonance (MR) angiography certainly belongs in this category. CT has become the an alternative modality of first choice, and it is also challenging pulmonary angiography as the gold standard. Direct visualization of patent pulmonary arteries and thromboembolic material is complemented by that of effects on the pulmonary parenchyma and right heart function; it also provides perfusion studies and MR-based flow measurement to assess hemodynamic compromise. Ventilation studies have long been a domain of nuclear imaging, and new techniques for the direct visualization of ventilation are emerging from recent developments in the field of MR imaging, for example, using hyperpolarized inert gases. New functional parameters of ventilation can be derived from these studies. For the diagnosis of metabolically active disease, such as tumor and pneumonia, CT offers very high sensitivity, for example, in screening for intrapulmonary nodules using low-dose CT and in the early detection of pulmonary infiltrates in high-risk patients. Especially for characterizing pulmonary nodules there is a need to combine nuclear medicine techniques, such as in positron-emission tomography.

Sideroelastosis pulmonum was first described by Ceelen (1931) and Gellerstedt (1939), mentioning an idiopathic type, but also vascular alterations in chronic pulmonary congestion. We analysed the question wether secondary sideroelastosis can be demonstrated also in lungs after previous welder dust exposure. Lung samples of 43 patients and deceased with various exposure periods to welder dust were investigated. On each sample, light microscopical, scan electron microscopical and energy dispersive x-ray micro analyses (EDX) were performed. In six samples (14%) vascular alterations in the area of denaturated elastic fibres with siderophilic "impregnation" were seen, corresponding to the features described by von Ceelen and Gellerstedt. Giant cell or granulomatous reactions, however, were present only in exceptional cases. This phenomenon was seen mostly in cases of marked local siderin deposits and reactive fibrosis. Using EDX, a number of metals characteristic for welder dust (Al, Ti, Cr, Ni) as well as iron were found in siderophages. No other metal than iron was detected in siderophilic elastic fibres using EDX. Causal pathogenetically, a correlation between increased iron uptake connected with welding particles and sideroelastosis of the vessel walls has to be established. However, in formal pathogenesis, other factors such as local perfusion disturbances due to fibrotic processes after exposure to harmful substances must be taken into consideration.

We report on a 43-year-old man with a primary sarcoma of the liver. The patient was admitted to the hospital for evaluation of dyspnea, abdominal pain in the right upper quadrant, diarrhea, and fever. Physical examination revealed hepatomegaly. Increased laboratory values were found for gamma-GT, LDH, CA 125, and NSE, but not for aspartate and alanine aminotransferase. Computed tomography presented a tumor in the right lobe of the liver. Venous cavography revealed a caval tumor thrombus reaching up to the right atrium. Major liver resection combined with replacement of the vena cava inferior was proposed, but before operation the patient complained about shortness of breath. Spontaneous fragmentation of the tumor thrombus with consecutive embolization of the lungs was suspected. Despite lysis therapy the patient died because of right ventricular failure. Autopsy revealed a tumor measuring 8 cm in diameter, which was located in the right lobe of the liver and invaded the inferior vena cava. Because of multiple tumor aggregates seen in the left and right main pulmonary arteries acute tumor embolization of the lungs was regarded as cause of death. Histologically the tumor was composed of bizarre giant cells surrounded by irregular spindle cells. The positive immunoreactivity pattern of the tumor cells for vimentin, lysozym, and CD68 justified the diagnosis of a malignant fibrous histocytoma (MFH) of the liver.

Purpose: This study compares contrast-enhanced 3D-MR angiography (MRA) of the pulmonary arteries with selective intra-arterial DSA in patients with chronic thromboembolic pulmonary hypertension., Materials and Methods: 20 patients preoperatively underwent a contrast-enhanced 3D-MRA of the pulmonary arteries at 1.5 T using the phased-array body coil. For MRA, we used a 3D-Flash-sequence after bolus timing. 2 radiologists analyzed the acquired image material in consensus with respect to the detection of central thromboembolic material and the visualization of the pulmonary arterial tree. Finally, the MR angiograms were compared with selective DSA images using surgical findings as the definitive standard., Results: MRA demonstrated central thromboembolic material, vessel cut-offs and abnormal proximal-to-distal tapering in all patients. Compared to DSA, MRA depicted the pulmonary vessels up to the segmental level in all cases, it was inferior to DSA in delineation of the subsegmental arteries (sensitivity 87%, specificity 100%). The central beginning of the thromboembolic occlusions seen at MRA corresponded to the beginning of the deobliteration procedure during pulmonary thromboendarterectomy in every case., Conclusions: Contrast-enhanced 3D-MRA of the pulmonary arteries enables the reliable detection of central thromboembolic material in patients with CTPEH. It also allows identification of those patients who may be treated surgically.

A case of a primary pulmonary leiomyosarcoma originating in the right pulmonary artery in a 61-year-old woman is reported. Patient's complaints were non-specific and after non-invasive diagnostics a chronic thromboembolic event was suspected to have occurred a long time ago. During a heart catheter investigation prior to planned surgical embolectomy a small specimen was taken from the pulmonary artery. Histological examination revealed malignant the tumour and by immunohistological staining its smooth muscle differentiation was confirmed. Complete resection of the tumour was achieved by radical surgery, including pneumonectomy and extensive resection of pulmonary vessels with subsequent implantation of a Goretex prosthetic. There was no evidence of recurrence or metastasis of the primary leiomyosarcoma of the pulmonary artery in a 14-month follow-up period after operation.

A 85-year-old woman was admitted to our hospital because of a presumtive diagnosis of pulmonary thromboembolism. The patient presented with a history of progressive dyspnoea and retrosternal pain. 3-4 weeks ago she had noticed a swollen left leg. On examination a 4/6-pansystolic murmur was found. An arterial blood gas analysis showed a reduced oxygen saturation. An electrocardiogram revealed deep S-waves in lead I and pathological Q-waves in lead III. The chest X-ray showed cardiomegaly, a pulmonary nodule and an ill-defined opacity inferioposteriorly. Ventilation-perfusion mismatch was demonstrated by lung ventilation-perfusion scanning. Transthoracic echocardiography showed pulmonary hypertension and tricuspid regurgitation. On the 20th hospital day the patient died from multi organ failure. Pulmonary thromboembolism secondary to deep venous thrombosis of the lower extremities was the most likely diagnosis. In view of the patients' history of night sweat, loss of appetite and weight loss a malignant process had to be taken into consideration. A tumor originating from the right ventricle, the right ventricular outflow tract or the pulmonary artery was compatible with the clinical picture of multiple pulmonary emboli. On autopsy a polymorph cellular sarcoma measuring 6 x 3 x 3 cm was found in the right ventricular outflow tract. Section of the lung revealed a single pulmonary metastasis and multiple thromboemboli of various age. Pulmonary artery sarcomas, as described in our case, are extremely rare. The prognosis is poor and often the diagnosis is only made on autopsy.

The flow directed balloon-tipped pulmonary artery catheter introduced by Swann and Ganz 1970 has made possible the measurement of filling pressures in the heart and is extensively used in operating rooms, in catheterization laboratories and in intensive care units. The rupture of the pulmonary artery is an uncommon complication associated with a high mortality rate exceeding 50 percent. Main symptoms are cough, hemoptysis, dyspnea and cardiac shock. Complications occur if guidelines for the safe use of the balloon-tipped catheters are not strictly followed: Excessive catheter manipulation, advancing the catheter tip too far peripherally and leaving the inflated balloon in the wedge position for long periods should be avoided. Patients with pulmonary hypertension as well as elderly and anticoagulated patients are at greater risk.

Unlabelled: OBJECTIVE AND CLINICAL FINDINGS: A 48-year-old woman was hospitalized because of haemoptysis. Until shortly before admission she had been on phenprocoumon after pulmonary embolism sustained 18 months previously. Six months before admission systemic lupus erythematodes (SLE) had been diagnosed and treatment with cortisone initiated. Physical examination revealed jugular venous congestion, tachycardia, dyspnoea on even minimal physical activity and pretibial oedema., Investigations: Lung scintigraphy showed a perfusion deficiency in the right lung, unchanged since a test 18 month before. Doppler echocardiography recorded an estimated pulmonary artery systolic pressure of 110 mm Hg. Angiography showed a fully patent superior vena cava and nearly complete occlusion of the main right pulmonary artery by a thrombus., Diagnosis, Treatment and Course: The haemoptyses ceased after 5 days of treatment with methylprednisolone, 130 mg daily for 5 days, reduced after 5 days to 80 mg i.v. every other day, plus cyclophosphamide, 50 mg daily by mouth. The pulmonary hypertension remained unchanged so that pulmonary thrombendarterectomy was indicated. Surgery revealed extensive mediastinal fibrosis and almost complete occlusion of the thick-walled right pulmonary artery by thrombus adherent to the wall. Histology showed vasculitis of the pulmonary arterial intima and of the small pulmonary vessels. After thrombectomy the pulmonary arterial systolic pressure fell to an remained at below 40 mm Hg. Phenprocoumon was continued (at an INR of 2.5-3.5) as was immunosuppressive treatment. The patient has remained free of symptoms and is able to be physically active., Conclusion: Pulmonary hypertension is a serious complication of SLE. Echocardiography is recommended for both the original diagnosis and serial follow-up, complemented by other imaging methods if indicated.

The frequency of pulmonary embolism in patients with deep vein thrombosis can be assessed by pathological-anatomical and by nuclear medical studies. The frequency of deep vein thrombosis in autopsies ranges from 23.7% to 62%, by inclusion of microscopic thrombi the frequency increases to 72%. In most cases the localisation of the venous thrombosis is bilateral. In cases of venous thrombosis the frequency of pulmonary embolism is 52 to 79.4%, if microscopic thrombi are included, the number is 87.8%. 7.8% to 78.9% of all pulmonary emboli are considered as cause of death or severely contributing to death. The fatal embolisms originate preferentially from iliofemoral thrombosis. About 1/10 of all emboli originate from V. cava sup. and the right heart. In a small number of pulmonary emboli the origin could not be detected. The rate of correct intra vitam diagnosis is low, not more than 11 to 25% of all pathological-anatomical proven emboli had a correct diagnosis during life. In fatal pulmonary embolism the correct clinical diagnosis was made in 1/3. In nuclear medicine studies pulmonary embolisms are searched for from the clinical suspicion or the diagnosis of deep vein thrombosis with the perfusion-/ventilation- or inhalation scintigraphy. Patients with deep vein thromboses showed in 38 to 57.9% pulmonary embolism. In 80% of all pulmonary embolism multiple perfusion defects (2 to 9 perfusion defects) were detected, the lesions were evenly distributed in both lungs. The frequency of pulmonary embolism in calf vein thrombosis was 46%, in leg vein thrombosis 67% and reached 77% if the pelvic veins were involved. It is remarkable that the majority of all pulmonary emboli (46.3% to 100%) showed no clinical symptoms. The knowledge about the high frequency of pulmonary embolism in patients with deep vein thrombosis can improve the diagnosis of pulmonary embolism. For the diagnostic process of pulmonary embolism the presence of acute deep vein thrombosis increases the pre-test probability (prevalence of more than 50%). Every positive test for pulmonary embolism will gain a very high post-test probability according to Bayes' theorem.