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Respiratory bronchiolitis (RB) is defined by the accumulation of pigmented macrophages in the lumen and wall of respiratory and membranous bronchioles of smokers. The aim of this study was to determine whether spontaneous pneumothorax was associated with a high prevalence of RB. Seventy-nine consecutive patients who underwent a surgical procedure (thoracotomy or thoracoscopy) for recurrence or persistence of primary spontaneous pneumothorax despite thoracic drainage were studied retrospectively. RB was found in 70 of 79 (88.6%) smokers operated for spontaneous pneumothorax. Associated interstitial pathological abnormalities were present in 53 of 79 cases (67.1%). In nine patients, the pathological lesions were severe and resembled desquamative interstitial pneumonia. Emphysematous lesions were present in about one-third of the patients. Although the possible pathophysiological consequences of respiratory bronchiolitis remain speculative, this study demonstrates the high prevalence of this pathological abnormality in patients with pneumothorax requiring surgical treatment.

Assessing 100 cases of arrhythmogenic right ventricular dysplasia causing unexpected sudden death, we stress two aspects of the disease, specially concerning pathologists: diagnosis value and limitations of endomyocardial biopsy and high frequency of the disease among unexpected sudden death in young adults. When the disease is identified genetic screening should be performed among patient's family members. The anterior right ventricular wall is very thin (partly or completely) and microscopic examination shows absence of muscle cells, replaced by normal fatty tissue. Associated lesions are often present: slight abnormalities of left ventricle, mucoid degeneration of auriculo-ventricular valves and recently described lesions of His conductive tissue. Apoptotic anomalies are now incriminated for agenesy or disappearing right ventricular muscle.

Two cases of pulmonary artery angiosarcomas are reported. Pulmonary artery tumor was diagnosed during exploratory thoracotomy in the first case. It was suspected on magnetic resonance imaging and confirmed by surgery in the second case. The therapeutic was a right pneumonectomy for the first patient and a tumor resection followed by chemotherapy and radiotherapy on cerebral metastases for the second patient. Both our patients died, 13 and 44 months after surgery. On the basis of histological and immunohistochemical findings both tumors were angiosarcomas. Ultrastructural examination in the second case revealed an endothelial differentiation. The review of 142 previously reported cases of pulmonary artery sarcomas and these ultrastructural findings suggest that pulmonary artery sarcomas may have multiple origins; leiomyosarcomas deriving from arterial smooth muscle; angiosarcomas deriving from the endothelium and undifferentiated sarcomas or "myointimal sarcomas" deriving from myointimal cells.

To assess the prevalence and thrombogenic role of anticardiolipin antibodies (aCL) in giant cell arteritis.Two hundred eighty-four patients with newly diagnosed temporal arteritis or polymyalgia rheumatica and 210 age- and sex-matched controls randomly selected from the general population were included in a multicenter, prospective case-control study. Blood samples were obtained at the time of diagnosis, data on initial clinical features were collected in a questionnaire, and temporal artery biopsy findings were reviewed by an experienced pathologist.Anticardiolipin antibodies were present in 20.7% of patients compared with 2.9% of controls (P = 1.45 x 10(-9)). The prevalence of aCL was higher in samples found positive for temporal arteritis than in those found negative on biopsy (31.2% versus 16.7%; P = 0.04), and was similarly higher in the biopsy-positive temporal arteritis group compared with the polymyalgia rheumatica and control groups. Although aCL were associated with thrombotic complications in univariate analysis, the positivity of the biopsy findings remained the only predictive variable in stratified analysis.In giant cell arteritis, aCL seem to function as reactive antibodies in relation to endothelial lesions.

The anatomic lesions of two cases of sudden death (one man and one 24 years old woman) and of one case of rapidly fatal myocardial infarction (51 years old woman) are described. The coronary lesions were exclusively thrombotic with no associated atherosclerosis or pre-existing arterial wall change. The thrombi were occlusive from the outset or were constituted in successive mural stages, the last being occlusive and giving rise to the clinical symptoms (sudden death and/or myocardial infarction). Several points are discussed. The concept of normal coronary artery must be confirmed by anatomic examination: coronary angiography is not sufficient for the diagnosis of dangerous atherosclerotic plaques which are not particularly stenotic, eccentric, lipidic and fragile. Thrombogenic factors are better understood and some may play a role in these cases of "primary thrombosis", by they of plasma or platelet origin. The trigger role of spasm cannot be ignored as some of the spastic and thrombotic factors are similar. The preferential coronary site of these thromboses may be explained by constitutional changes (arterial hypolasia with narrow lumen) or acquired lesions (repeated arterial trauma due to shearing by the movements of the underlying left ventricle transmitted to the epicardial coronary arteries).

The aim of this study was describe the anatomical features of coronary aneurysms and assess their role in causing death. Twenty clinico-pathological cases were recensed prospectively out of 8,920 autopsies (0.22%). The average age at death was 68 +/- 20 years (14 men and 6 women). Death was due to a cardiac cause in 90% of cases. The aneurysms were located on a single coronary artery in 14 cases, on 2 coronary arteries in 3 cases, and on all three arteries in 3 cases with a mean maximum diameter of 15 +/- 4.5 mm. Aneurysms of the right coronary artery were the most common (p0.01) and the largest (16.7 +/- 3.8 mm; p = 0.12) and circumflex arteries (12 +/- 3.1 mm, p = 0.03). The diameter of the coronary aneurysm was greater in the more elderly patients (r = 0.51 ; p = 0.022). The coronary aneurysms were diffuse in 10 subjects and localised in the other 10 (mainly fusiform and proximal). Only 3 aneurysms were directly associated with significant stenosis of the same artery. The aetiology was atherosclerosis in 18 cases, with associated extra-cardiac disease in 14 cases; the other 2 cases were dystrophic. The underlying anatomical abnormality constantly found was variable degrees of atrophy and destruction of the musculo-elastic elements of the media, a source of dilation. In 80% of cases, thrombosis of the aneurysm was directly or indirectly responsible for death. Coronary artery aneurysms are a rare finding and are frequently complicated by fatal thrombosis. Coronary angiographic detection of this condition characterised by the large size and absence of stenosis of the artery should lead to prophylactic antithrombotic therapy.

Intravascular ultrasound enables detection of the components of atherosclerotic plaques. The diagnostic value was assessed by ROC (receiver operating characteristic) curves on images acquired in vitro and correlated with the histological findings in 61 arteries. Five questions were asked of each operator; the reply was represented by a continuous variable in order to express all nuances of judgement. The area under the ROC curve, Az, was the criterion of performance (0.5 : chance response : 1.0 : all replies were accurate). Detection of plaque was satisfactory (Az = 0.89). The three layer appearance of muscular arteries was well recognised (Az = 0.94). The fibrous composition of a plaque was only just satisfactory (Az = 0.88) with 38.7% interindividual variability. The lipid composition of the plaque was poorly recognised (Az = 0.76) with large interindividual variability (52.8%) : hypoechogenicity was too ambiguous a sign from the acoustic point of view. A hypoechogenic zone must not be synonymous with a lipid plaque but a cellular zone. Calcium can almost always be detected (Az = 0.98) with a very low interindividual variability (10.7%), fibrohyaline progression of some plaques can be confusing. The authors present a more objective description of endovascular ultrasonographic images. They conclude that the diagnostic performance of 30 MHz intravascular ultrasound is satisfactory but several limitations are apparent in the interpretation of images, especially hypoechogenic zone and hyper-reflective zones with high attenuation.

We report a case of tracheal leishmaniasis with stenosis in a 52 year-old female originative from the Gard region (South of France). The unusual localization acknowledges for the difficulties met in setting the diagnosis which was established through cytological examination of bronchial brushing. Though rare, leishmaniasis infection must be suspected in all cases of mucosal lesions occurring in patients living in endemic areas.

The outcome of 75 cases of post-radiotherapy pericarditis severe enough to lead to surgery, was reviewed from 1970 to September 1995. Four clinical forms were identified: acute pericarditis resistant to medical treatment (12 cases), large chronic pericardial effusions resistant to medical treatment (16 cases), chronic, compressive effusions (35 cases) and signs of pericardial constriction (12 cases). The medical conditions irradiated were Hodgkin's disease (41 cases), followed by carcinomas (27 cases), malignant lymphomas, thymoma and seminoma. Forty-three deaths were observed during the study period (only 4 unrelated to the neoplasia or radiotherapy) after a mean interval of 7.4 years after radiotherapy and 2.5 years after surgical biopsy: 25 were related to cardiopulmonary complications of radiotherapy: 14 were due to recurrence of the malignant disease or the appearance of another tumour. Fifty-two pericardial effusion drainage procedures and 28 pericardectomies were performed, due to the necessity for reoperation. The authors have tried to determine therapeutic indications based on recognised physiopathological data and the anatomical aspects of the four different clinical forms. The prognosis is affected by post-radiotherapy fibrosis which extends beyond the pericardium to involve other cardiac structures and the pulmonary parenchyma. However, it must be emphasised that the initial survival of all these patients was due to the radiotherapy.

One hundred patients operated for left atrial myxoma in the same surgical department underwent clinical and anatomical assessment at long-term from 1959 to July 1995 (66 women and 34 men, average age 52.2 years). The clinical presentation was related to mitral valve obstruction in half the cases (dyspnoea, cough, pulmonary oedema), the presentation in the other half of cases being very variable. The widespread use of echocardiography has relegated other investigations to a subsidiary role: auscultation, radiology, ECG (9 cases diagnosed by echocardiography performed for another indication). Serious complications of left atrial myxoma include systemic embolism : 37 cases out of the 100 in this series, including 10 plurifocal but mainly cerebral (19 cases including 11 isolated cerebral emboli). Surgical treatment is well established, should not be deferred and gives excellent results (2 early postoperative deaths out of 100 cases in the early years of the study). There were 6 cases of recurrences including 3 cases of Carney's syndrome. Clinico-pathological correlations showed that mitral stenotic effects occurred when the tumour diameter exceeded 5 cm and embolism was associated with tumours having multiple villositi. Histopathological analysis distinguished between active and inactive tumours, differentiated or not, and enabled the elaboration of hypotheses on the rate of growth of the tumour and on the absence of true metastases. Histopathological techniques also show the presence of lymphoplasmocytic infiltration, the sign of secretion of interleukin 6 by the myxoma, a cytokine involved in the general inflammatory process and which explains the unusual clinical presentation sometimes observed.

Seventy one cases of apparently idiopathic chronic pericardial effusion were systematicaly followed up. There were 3 inclusion criteria: patients had to be symptomatic with radiological cardiomegaly without tamponade or an acute onset; the effusion had to be stable for more than 3 months despite medical therapy at the time of surgery; the parietal pericardium removed at surgery had to be of normal histopathological appearances. Cases with a discernable classical cause of pericardial effusion were excluded: previous mediastinal radiotherapy, connective tissue diseases, malignancies, chronic infection or a recent episode of acute pericarditis. There were 9 deaths during the first five postoperative years, one of which was related to the pericardial disease and surgery. By definition, no cause was found but in one third of cases a relationship between the pericarditis and other disease processes was possible. The management of these large pericardial effusions resistant to treatment without any apparent underlying pathology and in which echocardiography only shows a large effusion should be surgical with drainage of the pericardial cavity, allowing exclusion of aetiologies which are non-identifiable by other methods, with a negligeable operative risk even in elderly patients and good long-term clinical results. This recommendation is based on systematic follow-up of over 5 years in 57 of our 71 cases; after drainage, only one of the nine deaths observed was related to the pericardial disease itself and 59, subjects (83%) had no further symptomatic pericardial disease.

In order to determine the results of cardiac retransplantation 38 cases were reviewed (42 transplantations; 4 patients underwent 3 transplantations). Clinical indications included acute rejection and infection before and after retransplantation: the explanted heart was sent for anatomopathological study. The indications were dominated by coronary artery disease of the graft (26 cases) with an average of 45.3 months between the two transplantations and a long-term survival comparable to that of primotransplantation. Conversely, in the other indications of retransplantation, the delay was much shorter (less than 12 days in half the cases) and the results were poor (9 deaths out of 12 cases). Therefore, coronary disease of the graft would seem to be the only justified indication for retransplantation at it associates stable haemodynamic conditions and therapeutic impregnation comparable to the primotransplantation.

Sarcoma of the common pulmonary artery are rare malignant tumors which can mimic pulmonary embolism. In the case presented here, the inaugural signs were particularly misleading: multiple pulmonary lacunae on computed tomography. The unusual aspect and asymmetric localizations at pulmonary angiography then suggested the doubtful nature of the embolism etiology. Magnetic resonance imaging findings suggested the diagnosis of sarcoma of the pulmonary artery. Certain diagnosis was obtained at pathology examination of the surgical specimen after thoracotomy. A malignant fibrous histiocytoma was identified. Curative resection was not possible and chemotherapy was performed. Unusual parenchymal lesions were then evidenced on the radiography. Better and better magnetic resonance imaging criteria are described in the literature and help distinguish between thromboembolism and sarcoma of the pulmonary artery. Follow-up of the clinical course is thus improved. It is nevertheless necessary to evaluate intravascular extension to determine whether curative surgery is possible.

The aim of this study was to search for a cardiac cause of death after an otherwise negative medico-legal autopsy in 1,000 adults under 65 years of age with no previous history, who suffered a sudden death. Four hundred and seven coronary causes were established with 152 negative autopsies and 441 other cardiac lesions. These 441 cases comprised 193 cardiomyopathies, 106 His bundle lesions, 50 partial forms of Uhl's anomaly (or arrhythmogenic right ventricular dysplasia), 14 isolated mitral valve prolapses, 22 chronic valvular lesions, 8 myocarditis, 16 pulmonary embolisms, 11 chronic cor pulmonale, 11 aortic dissections and 10 atrophic fibrous plaques of the left ventricle, sequellae of thoracic trauma. His bundle lesions (isolated and associated) were observed 299 times out of 1,000 cases and mitral valve prolapse 125 times. Age and gender, circumstances of sudden death, the weight of the heart, are reported for each group of conditions. The frequency of associations, especially of His bundle lesions, suggests that they may have a potentialising effect on the genesis of the fatal arrhythmia.

A 31 year old right handed woman presented with acute onset of aphasia which cleared over two days. CT-scan showed a left middle cerebral artery infarct within Wernicke area. Initial transesophageal two-dimensional echocardiography disclosed a mitral valve lesion suggesting a thrombus. She was discharged on oral anticoagulant treatment. A second stroke occurred ten months later involving left lenticulo-striate arteries area. Echocardiography remain unchanged. Subsequently, giant Lambl's excrescences of mitral valve was confirmed by operation and pathologic examination. The majority of patients with Lambl's excrescences are asymptomatic. However surface thrombus is common with this tumors which reposant a potential us for cerebral embolization. These tumors should be operated since complete excision is the only definitive means of eliminating the source of recurrent embolization.

We report a retrospective study of the pathological features in 69 primary pulmonary non-Hodgkin's lymphomas which have previously been clinically reviewed. The tumours consisted of 61 (88%) low-grade and eight (12%) high-grade malignant lymphomas. Fifty-four of the low-grade malignant lymphomas were MALT lymphomas. Lymphoepithelial lesions were observed in bronchial, bronchiolar and alveolar lining. All tumours were composed of nodules, forming a lymphangitic pattern at the periphery and a confluent central mass. Invasion of pleura and vessels was often seen but this without any consequence on survival. Granulomas were found in 20% of cases. Six of the eight high-grade tumours were centroblastic and another two were B-cell lymphomas of undetermined type. In four cases, associated areas of low-grade malignant lymphoma with lympho-epithelial lesions indicated a preexisting MALT lymphoma. Clinical data suggest that limited surgery or non-aggressive chemotherapy can provide long-term survival in patients with such slowly developing neoplasms. However, non-invasive diagnostic methods need to be developed.

We report a case of acute fatal fibrosing silico-proteinosis occurring in nine months in a young woman who had voluntarily inhaled scouring powder. We recall the reports in the literature of the subacute or chronic forms associated with systemic diseases occurring after such inhalational practices. We recall the need for specific questioning and to complete the study, the need for microscopy and electron-microscopy associated with X-ray defraction, either on the alveolar lavage fluid or on a lung biopsy.

This article reports the difficulties of the diagnosis of endocarditis, especially when blood cultures are negative. It emphasizes the respective roles of the microbiology and pathology laboratories for the processing of cardiac valves removed by surgery. It clarifies the morphological characteristics of the endocarditis.

Today, the pathology of large and medium-sized arteries is in most part considered as degenerative or inflammatory. The role of infection has been preponderant (syphilis) but has become quite modest now, restricted to infectious aneurysms. According to certain observations, infections may participate in initiating arterial inflammation, whether it be specific (Kawasaki's disease, Takayasu's arteritis, coronary artery disease of cardiac grafts) or less so ("plain" atherosclerosis). Suspected microbes (herpes viruses, Chlamydia pneumoniae, etc.) would damage the arterial wall either directly by infecting it, or indirectly by provoking an autoimmune reaction against some of its components (e.g. heat shock proteins). These hypotheses are worth serious consideration because, if established as correct, they would modify radically our etiologic, therapeutic and prophylactic conceptions of arterial diseases, including of course the main one, atherosclerosis.

The authors emphasise the role of the pericardium in the post-operative complications of cardiac transplantation: its role is as important as it is underestimated in its frequency, severity and the pathogenic discussions that it induces. This study, based on a retrospective analysis of 191 anatomo-clinical cases (156 autopsies and 35 retransplantations) of patients undergoing cardiac transplantation before the 1/03/1993 including macroscopic and histopathologic analysis of the pericardial lesions, histochemical analysis of the lymphocytic populations, and analysis of associated coronary and myocardial lesions. The results were classified as early or late (after the 75th postoperative day) complications. Early complications included 27 suppurating mediastino-pericarditis (13 aspergillosis and 14 bacterial), 16 autonomous non-infective pericardial complications (6 haemopericardiums, 6 organised compressive haematomas, 4 early constrictive pericarditis) and 9 lymphocytic epicarditis associated with acute myocardial rejection. Late complications included one common constrictive symphysis and 14 reactivated epicarditis associated with transplantation coronary disease (chronic rejection) with associated lymphoplasmocytic nodules and a myocardial vasculitis. These observations suggest two important conclusions: 1) the need for active diagnostic measures to detect pericardial complications in cases of cardiac transplant dysfunction in the early postoperative period with no signs of acute rejection on myocardial biopsy: the presence of epicardial lesions on biopsy should be taken into account and not just assumed to be a postoperative epiphenomenon; 2) the presence of epicarditis due to reactivated epicarditis may cause inextensible hardening of the epicardium which adds to the effects of restrictive cardiomyopathy of chronic rejection.

Ten pericardial mesotheliomas (8 of which had associated unilateral pleural involvement) were observed over a 22 year period in subjects over 50 years of age. The diagnosis was only confirmed several months after the presenting symptoms (shortness of breath, chest pain), usually by histological studies of pericardial biopsies performed during construction of a pleuro-pericardial window because of tamponade or of pleural biopsy in cases of pleuro-pericardial disease. There is no specific diagnostic feature and even modern imaging methods are unable to distinguish mesothelioma from pericardial tuberculosis. In 7 cases, there were large haemorrhagic pericardial effusions. At present, there is no effective treatment for mesothelioma and the physician's goal is to make the patient's short survival time as comfortable as possible with respect to the severe pain and recurrent pleuro-pericardial effusions. The pericardial mesothelioma is rare (less than 1% compared with 96% pleural and 3% peritoneal localisations) and possibly related to exposure to asbestos, at least in those cases with associated pleural involvement. The authors underline the utility of histological analysis of the utility of histological analysis of the pericardium if only to establish the diagnosis of mesothelioma and to enable administration of curative treatment of other pathologies (tuberculosis, malignant lymphoma) with identical clinical presentations.

Visna-maedi virus is a lentivirus that causes a chronic disease in sheep affecting, among other organs, the lungs. Interstitial pneumonitis is similar to that in man associated with the infection by the human immunodeficiency virus type-1. We have compared the pathological features of lungs of sheep naturally infected with visna-maedi virus with the results obtained from bronchoalveolar lavage and virus isolation. Semi-quantitative grading of the lesions was performed on 147 sheep lungs obtained from the slaughterhouse. Seventy-seven were macroscopically and histologically normal, 39 had typical lesions of interstitial lung disease (maedi), and 13 had minor lesions of the same type. Eighteen of the affected lungs were heavily infested with parasites. Of these parasite-infected lungs, 9 showed typical maedi lesions and 4 showed minor lesions; parasite infection had no obvious effect on the development of maedi. In keeping with pathological findings, bronchoalveolar lavage disclosed an alveolitis process in the maedi lungs with increased macrophage, lymphocyte and neutrophil numbers. Cytopathic virus was detected from alveolar macrophage coculture with fibroblasts more often from maedi lungs (10/12) than from normal lungs (9/39). Electron microscopy of bronchoalveolar lavage cocultures revealed typical lentiviral particles. Animals with minor lesions may be at an early stage of the disease.

Infective endocarditis remains a common condition for pathologists interested in cardiology who fortunately examine more infected valves excised surgically (66 in 1992) than observed at autopsy after death from this condition (2 in 1992). The authors discuss the elementary valvular lesions (ulceration and vegetations), the severity of which affects the prognosis, and the special aspects of these ulcerating vegetations with respect to their location (aortic, mitral, pulmonary and tricuspid), to the type of underlying valvular disease (rheumatic, myxoid or calcific) and infecting organism. The extravalvular complications are then reviewed: annular abscess (and possible extensions), purulent pericarditis, parietal endocarditis, myocarditis and coronary embolism. The authors attempt to answer questions about infective endocarditis from the pathologist's viewpoint: the difference between acute and subacute endocarditis, the reality of infective lesions of "healthy hearts", the role of the pathologist in the detection of pathogenic organisms, the evolution of lesions after sterilisation. The particular situation of prosthetic valve (biological or mechanical) endocarditis is treated in detail. The role and possibilities of surgery, the value of which is now universally accepted (the mortality of severe infective endocarditis has been lowered from 50-60% to 10-20% by a good operative strategy) are emphasised throughout.

The aim of this study was to attempt to determine which anatomical lesions were found late after cardiac transplant and could be fatal. A series of 44 autopsies, beyond a 6 month survival time, revealed the types of cardiac and extra-cardiac lesions encountered. They were viewed in the context of clinical findings. Three lesion patterns accounted for more than 9 cases out of 10: coronary disease affecting the transplant (accelerated atherosclerosis or chronic cardiac rejection: 48% of cases), infectious lesions (29%), more than half of which were due to aspergillosis, and neoplasia (14%), favourised by immune-depression. Three results show that graft coronary disease is currently the essential long term problem in the many patients who survive the early postoperative period of cardiac transplant.

Intravascular ultrasound is a new method of visualizing details of vascular pathology, providing (real time) high resolution images of vascular walls. Most of the research on the technique has explored its qualitative and quantitative capabilities to improve the assessment of atherosclerotic vascular disease in vivo. Intravascular ultrasound differs from angiography and angioscopy in its ability to penetrate below the surface of the vessel lumen, demonstrating specific appearances of the distribution and composition of plaque. Image analysis is operator dependent. Although this technology is very promising limitations such as artefacts and loss of image quality in heavily calcified vessels hinder its use. There is hope that this imaging technique may ultimately improve the results of endovascular interventions.

International audience; The present study is a prospective examination of the relationship between platelet aggregation and the occurrence of graft failure in a single cohort of heart transplantation (HT) recipients. One-hundred-and-twenty-four patients underwent platelet function study and were then followed for 1 to 24 months (mean 6.7 months). There were nine re-transplantations and 13 deaths (11 related to ischaemic events, and two others). In 15 patients, pathologic examination confirmed or revealed that recent acute myocardial infarction was the obvious cause of the graft failure. In five patients, myocardial fibrosis related to severe and diffuse coronary disease was the only microscopic finding. In the last two patients, the cause of the heart failure was not clearly identified. In recent myocardial infarction there was a high incidence (14/15) of coronary thrombi. Thrombi were multiple, disseminated in the coronary tree end of different age. Their presence at autopsy or after explantation was associated with an enhanced ex vivo platelet aggregability as compared with patients without coronary thrombi (n = 8): 43.3 +/- 1.7% of maximal aggregation vs. 34.4 +/- 2.4 (P = 0.006) and 48.4 +/- 5.2 vs. 22.6 +/- 4.9 (P = 0.003) for the primary and secondary waves of ADP-induced aggregation. These results suggest that thrombosis and platelets may play a major role in the process of accelerated coronary artery disease after HT.

In order to determine the responsibility of coronary artery disease in unexpected sudden death (without known or treated cardiac disease), the authors studied 1,000 cases of "natural" unexpected sudden death in subjects under 65 years old in whom the autopsies were performed in Medico-Legal Institutes and in whom all non-cardiac causes had been excluded. Macro- and microscopic examination of the heart detected 848 potentially lethal lesions (152 autopsies failed to show the cause of death) of which 407 cases corresponded to coronary disease (atherosclerosis in 340 and other disease in 67 cases). The atherosclerotic lesions could be classified in 3 anatomical groups: 75 isolated recent coronary thromboses; 75 recent coronary thromboses associated with multivessel stenoses; 190 coronary stenoses (75%) without thrombosis (139 triple vessel, 31 double vessel and 20 single vessel diseases). The presence of a recent myocardial infarct, the cardiac mass and the circumstances of sudden death were determined in all cases with respect to the three pre-defined anatomical groups. This study, though confirming the high prevalence of coronary artery disease, evaluates it at a lower level than previous reports in the literature which takes into consideration "unexpected sudden death" of known and treated coronary patients, and also has allowed identification of other potentially lethal lesions (such as structural abnormalities of the His-Purkinje system) which are undetectable even with sophisticated paraclinical investigations.

Between 1969 and 1992, eighty cases of malignant pericarditis were studied by thoracotomy and biopsy, which made it possible not only to assert the diagnosis but also to dry the pericardial effusion by creating a pleuro-pericardial window. In 75 cases a cardiac tamponade of varying severity required surgical heart decompression. Pericarditis revealed the neoplasia in 53 cases and complicated a known cancer in 27 cases (23 carcinomas, 2 malignant melanomas included). In 63 cases (2 metastatic malignant melanomas, 7 sarcomas and 54 carcinomas) the disease was rapidly fatal. On the other hand, 17 patients with certain types of neoplasia revealed by pericarditis (12 with malignant lymphoma, 5 with non-encapsulated thymoma) have survived under an appropriate treatment. In addition to curing the immediately life-threatening tamponade, thoracotomy with pericardial biopsy determines the nature of the lesion and, consequently, its specific treatment.

Peripheral arteriolitis of the legs from the 4th postoperative day after cardiac transplantation caused ischemia of the feet and required bilateral transmetatarsal amputations on the 28th postoperative day. The causal condition was cytomegalovirus infection, confirmed by rising IgM antibody titres, the detection of a viraemia on the 33rd postoperative day, and, above all, the presence of cytomegalic inclusion bodies in many endothelial arteriolar cells of the amputated limbs. The patient died four months after transplantation and autopsy revealed major lesions in all the distal coronary vessels of the graft which occluded over 4/5ths of the coronary lumens. An aspergillus septicemia was the direct cause of death.

Complications due to infection by aspergillus and favorized by therapeutic immunodepression are a significant cause of mortality after cardiac transplantation, but are only rarely diagnosed before autopsy. A detailed description of the anatomical lesions and their localisation might help the interpretation of symptoms observed in vivo. In a series of 147 autopsies subsequent to cardiac transplantation, aspergillosis appeared as the cause of death in 26 cases. Three anatomoclinical presentations could be identified: early post-operative septic pyohaemia (14 cases), delayed septic pyohemia (7 cases) and forms with predominantly pulmonary involvement (5 cases). In the first category, cardiac lesions are the most evident with myocardial micro-abscesses and intracardiac masses of aspergillus presenting a variable risk of embolism. Pulmonarytarget lésions(infarctus associated with a variable degree of aspergillus arterial thrombosis) were present in all cases. Visceral involvement completes the picture; the more frequent lesions being acute pancreatitis (9 cases), infarctogenic aspergillus emboli of the kidneys (11 cases), the spleen (9 cases) or the brain (6 cases). In 8 subjects, microscopical examination revealed other germs associated with aspergillus. The serious consequences of aspergillus infection after cardiac transplantation imply that every preventive precaution should be taken, especially in avoiding possible contamination of the air of the operating room by the installation of filters and the regular control of airways and air conditioning systems.

We studied 70 patients with biopsy-proven pulmonary non-Hodgkin's lymphomas without extrathoracic involvement or mediastinal adenopathy to determine the clinical, imaging, and endoscopic features of this condition in a homogeneous series. In low-grade (LG) lymphomas, symptoms were cough, dyspnea, chest pain, hemoptysis. Imaging features consisted of localized alveolar opacities, infiltrative diffuse opacities, atelectasis, and pleural effusions. Inflammatory changes of the mucosa were present in some patients, leading to bronchial stenosis in 7; biopsies showed lymphomatous infiltration in 12. Prognosis of LG lymphomas was excellent, with 93.6 percent survival at five years. High-grade lymphomas differed from LG lymphomas principally by a more aggressive course and a worse survival. Inflammatory changes occurred in seven of nine cases leading to stenosis in two, and biopsies showed lymphomatous involvement in five. The profile of primary pulmonary lymphomas in this study could help clinicians consider this condition and prompt them to evaluate new diagnostic tools.

The anatomical lesions of three subjects suffering from pulmonary histiocytosis X and treated with lung transplantation were studied in order to detail the changes occurring in late stages of the disease; the early stages are known on account of lung biopsies done with a diagnostic aim. All the subjects were, at the time of transplantation, in a precarious clinical state and were practically bed-bound as a result of their dyspnoea with a history of histiocytosis X which had been known for 9, 7 and 3 years respectively; one patient had a cardiopulmonary bypass and two unilateral lung transplant. The results of the histological study produced data which was slightly discordant from the communly held ideas; the de Langerhans cells persist in large numbers and after many years of the evolution of the disease, even though other elements of the granuloma have practically disappeared. Fibrosis is absent playing no role in the genesis of the respiratory failure. The lesions are destructive, leading to a disappearance of the alveolar capillary bed which is the source of the dyspnea. The honeycomb cavities to the destroyed lung are varied in nature: most correspond to the disappearance of the dividing walls of the alveoli or the pulmonary acinus, some others related to dilated terminal bronchioles and finally others to hollowed out granulomas which have become cavities. The peculiar characteristic of the lung allows for destructive cavities to be produced by the airflow, submitted to a process of auto-aggravation by the elastic forces and the tension of the pulmonary parenchyma.

The patient reported here had no particular past history. He presented with effort dyspnea, jugular turgescence and positional vertigo suggestive of a superior vena cava obstruction syndrome. Bronchial fibroscopy was normal, but chest X-ray, CT scan and phlebography of the superior vena cava revealed an anterior mediastinal tumour compressing the superior vena cava. The tumour was excised with replacement of the vena cava by a PTFE n. 16 graft. Pathological examination showed this to be an inflammatory tumour by fibrosis with plasmocytes. This plasmocyte fibrosis is histologically benign and becomes malignant only when complications occur as a result of its spread.

The results of 100 consecutive autopsy studies performed since the introduction and use of cyclosporine (1984 to 1991) in patients who died less than 2.5 months after cardiac transplantation were analysed to try to prevent this type of lethal damage. The lesions were complex but the causes of death may be classified as follows: 44 infections (20 aspergillosis, with 13 septicaemias and 7 predominantly pulmonary complications, 15 severe lung infections, 9 other infections including 7 pyogenic mediastino-pericarditis), 12 acute myocardial rejects, 14 pulmonary arteriolitis reflecting the fact that pulmonary resistances affect the results of cardiac transplantation, 13 non-infectious pericarditis, 17 immediate postoperative deaths (incompetent graft, DIVC). In the discussion, the authors underline the importance of pericardial damage, the direct cause of death in 13 cases but also present in most cases of infection when sometimes clinically confused with the diagnosis of "acute reject". Acute pancreatitis (over 10% of cases) were often labelled "septicaemic shock". Pulmonary involvement is one of the commonest complications related to infection and changes due to passive pulmonary hypertension related to the causal preoperative disease, by silent pulmonary embolism during the 3 months of cardiac failure before surgery and DIVC. Infection was the cause of death in nearly half of the early fatalities, and aspergillosis was particularly common whereas systematic prevention with sulfadoxine-pyrimethamine has eliminated pneumocystosis for example. The management of immuno-depression varies from centre to centre and this is also a factor in the incidence of anatomical complications.(ABSTRACT TRUNCATED AT 250 WORDS)

The aim of this study was to define the presence, histological types and extent of bronchiolitis obliterans (BO) after lung transplantation and to discuss the place of bronchiolitis obliterans in the progressive obstructive ventilatory disorder observed in some patients and considered to correspond to "chronic lung rejection". The histological lesions were studied in 16 subjects surviving for more than one month after lung transplantation or heart-lung transplantation: 12 autopsies, 3 surgical biopsies and one lung explanted for retransplantation. Thirteen subjects presented lesions of bronchiolitis obliterans: 5 cases of granulomatous BO, 6 cases of proliferative BO, one acute necrotising BO and one tuberculous granulomatous BO. Three of these patients presented an obstructive ventilatory disorder: one death on the 213rd day (acute necrotising BO), one retransplantation on the 672nd day (granulomatous BO), one death 53 days after surgical biopsy on the 247th day (proliferative BO). Two patients undergoing surgical biopsies with lesions of proliferative BO are still alive (cases 15 and 16) and do not present any signs of obstructive ventilatory disorder. This study suggests the existence of various histological types of BO in transplanted lungs, which is not exclusively of immunopathological origin, that infections and various inhalations also play a role and that alterations of pulmonary structures other than bronchioles are also involved in the pathogenesis of the obstructive ventilatory disorder (functional destruction of the transplant), corresponding to "chronic lung rejection".

The authors present the pathologist's view of Uhl's anomaly based on 100 cases recorded over 31 years, 89 of which came from medico-legal autopsies following sudden unexpected death in young subjects. The anatomic diagnosis is made by macroscopic examination of the whole heart in diffuse forms (parchment heart) and from transverse sections of the mid third of the ventricle in incomplete forms, showing absence of myocardium in the juxta-septal anterior wall of the right ventricle. The wall entirely made up of adipose tissue, may retain its normal thickness. On microscopy, normal adipose tissue is observed between the epicardium and endocardium, sometimes with islets of myocardium dispersed in the subendocardial layer and with, in about half the cases, subendocardial bundles of non-hyalinized collagen without any inflammatory cellular infiltration. Other structural histopathological lesions may complicate the malformation, involving the intraseptal arterioles or the conductive system. In addition to the 89 cases of sudden and unexpected death before the age of 50 (preceded by some modification of the patient's life style in 29 cases), 11 cases were symptomatic and 5 were transplanted with a good result. The clinical diagnosis of Uhl's disease is important because of its consequences: ventricular tachycardia and possible sudden death, more rarely cardiac failure with age. Histological analysis should take into account the constant presence of adipose tissue in the right ventricle, and should only interpret excessive quantities with associated myocytic aplasia as pathological.(ABSTRACT TRUNCATED AT 250 WORDS)

Polyaneurysmal dystrophy is a novel form of arteriopathy which specific clinical, angiographic, anatomic and surgical features which distinguish it clearly from multiple atheromasclerotic aneurysm. It should be considered to be a local, multifocal accentuation of megadolicho-arteries, which constitute the lesions during the early stages of the disorder (fairly general elongation of the elastic arteries, with thin walls and regular increase in the caliber and multiple tortuousness). Arterial angiography identifies polyaneurysmal dystrophy; in the context of a twisted and sinuous system of large arteries, multiple spindle-shaped aneurysms can be distinguished which are frequently bilateral and symmetrical. The usual sites are the trunks of the aortic group and internal carotid, the ileo-femoral trunks and terminal aorta. The progress of the disorder is characterized by the possibility of rupture or thrombosis (particularly in the subcrural territory). The treatment is always surgical. The indication for surgery is inevitable in cases of severe ectasia, but may be avoidable in extensive forms of megadolicho-arteries with no clearly defined aneurysm: annual ultrasound monitoring is then called for. The disorder is of constitutional origin (and totally unrelated to atherosclerosis). Delayed dilatation of the aneurysms is due to the hemodynamic forces brought to bear on the fragile walls over a life-time. Multiple aneurysms occur mainly between the ages of 50 and 70 years, with a predominance of aorto-ileac sites in men, even though these subjects do not show any general elastic dysplasia. Half of the 45 patient undergoing surgery were hypertensive.

Accelerated atherosclerosis of cardiac grafts is one of the factors limiting long-term survival after cardiac transplantation. The authors report the case of a patient who had a cardiac arrest associated with severe atherosclerosis 18 months after transplantation. The severity of the coronary lesions was underestimated by coronary angiography. An ergometrine test induced coronary spasm, a phenomenon which has only rarely been observed in transplanted hearts. The patient died one month later despite calcium inhibitor therapy. Autopsy revealed very severe triple vessel disease. This case illustrates the possible rapid evolution of coronary artery disease in cardiac transplant recipients, the difficulty in evaluating the severity of the lesions by coronary angiography and the additional possibility of observing coronary spasm in these cases.

The authors report two new cases of spontaneous dissection of the coronary arteries in women under 50 years of age without any predisposing factors and responsible for sudden death. One patient had dissection of the left anterior descending artery and the other had dissection of the left anterior descending and right coronary arteries. Special histological stains allow demonstration of abnormalities of the elastic tissue of the media in both cases. In the first case, a plaque of atheroma was present opposite the site of dissection but did not seem to be causal. In the second case, severe periadventitial cellular, infiltration of unknown origin was observed. This pathology of the young woman, often occurring in the postpartum period, is as rare as it is unpredictable.

Twenty anatomico-clinical cases of chronic cardiac rejection (accelerated coronary disease in heart transplant) consecutive to heart transplantation were studied with the view of obtaining detailed information on the anatomical features of coronary lesions, such as histopathological alterations, modalities of their diffusion to the 3 epicardial trunks and to distal intramyocardial branches, thrombotic complications and their consequences: massive (infarct) or disseminated myocardial ischaemia. The lesions observed were correlated with the corresponding coronary angiographic images, and an interpretation of the aetiopathological factors was attempted. Within a few months or years, the coronary lesions are found to progress towards very diffuse circumferential atherosclerous alterations where the plaques are clearly less individualized than in common atherosclerosis but thrombosis is frequent and multifocal in 50 percent of the cases. This produces a restrictive type of ischaemic cardiopathy which is painless since the heart is denervated, resulting in cardiac failure (11 cardiectomies for retransplantation, 9 autopsies) with coronary angiography tending to underestimate the importance of coronary damage. The most original aetiopathological factors seem to be arterial inflammation of immune origin, viral infections facilitated by immunosuppression and platelet hyperactivity, but their respective importance could not be accurately determined in this study.

In a series of 100 patients with intracardiac myxoma, the lesion was in the right atrium in 10 cases. The authors attempt to define the particular features of right atrial myxoma in terms of clinical aspects, outcome and pathology. The diagnostic difficulty raised by complex clinical pictures (including right ventricular failure, pseudopericardial forms and forms with a predominance of systemic problems) has now been resolved by imaging and, above all echocardiography. Consequences for the right side of the heart may involve the pulmonary artery with myxomatous emboli causing multiple fusiform aneurysms and pulmonary hypertension, or, more rarely, the tricuspid (with need for valve replacement), vena cava (Budd-Chiari syndrome) or atrial septum (right/left shunt). Alongside systemic problems due to interleukin 6, erythrocyte abnormalities are a new feature. Frequent etiological inclusion in a Carney complex explains recurrences and multi-cavity forms. The dominant pathological feature is tumor calcifications, no doubt as a result of the long presymptomatic phase of myxoma.

We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.

We studied a retrospective series of 25 sub-pleural fibromas (benign localized pleural mesotheliomas) of which 23 where excised, collected over a period of 20 years. The current series confirms the excellent prognostic of these rare tumors. They are sometimes bulky, but not infiltrating and discovered by systematic chest X-ray. Modern imaging using CT-Scan and nuclear magnetic resonance can suggest their diagnostic which is confirmed by histopathologic analysis of the usually pediculate and easily removed tumor. Transthoracic needle aspiration is generally of little help for diagnosis. The histopathologic diversity of the tumor is rather confusing but characteristic features can be found with fusiform cells ans collagen stroma. Immuno-histochemistry is more helpful for diagnosis and histogenesis recognition than electron microscopy.

The term "aortic aneurysm" applies to numerous conditions with extremely varied anatomical and aetiological features, which consist of more or less complete and localized destruction of the aortic wall components resulting in a sacciform or fusiform distension induced by blood pressure, with loss of parallelism between the sides of the aorta. The most frequent aneurysms, leading to numerous cardiovascular operations, are encountered mainly in middle-aged men. These aneurysms complicate the infra-renal terminal atherosclerosis of smokers or the parietal elastic dystrophy of the ascending aorta, where a few subjects in their twenties with Marfan's disease join the older men, and which clinically presents as severe aortic incompetence. The aneurysm of syphilis and its destructive effect on thoracic structures is a thing of the past with a purely historical interest. Dissecting aneurysms--which should be called incomplete internal aortic disease with delamination of the media--remains a fearsome disease in subjects over 50, despite advances in surgery. Rare types of aneurysm (traumatic, infectious, or local accentuation of megadolichoarteries, aortitis or connective tissue dysgenesis) complete the list.

The clinician confronted to the diagnosis of infiltrative lung disease has to consider a wide spectrum of aetiologies. Among them, diffuse fibrosing interstitial lung disease is characterized by the deposition of collagen within alveolar structures. Either acute alveolar injury or chronic inflammatory alveolitis can induce pulmonary fibrosis. Basic research investigates the cells, the cytokines, and the extracellular matrix involved in the perpetuation of the fibropoliferative process.

The results of a series of 930 coroner's autopsies were analysed retrospectively with the aim of determining the incidence and cause of sudden death during sports activities. Twenty three cases are reported (2.57%) of autopsies of individuals who otherwise had no traumatic lesion and no cerebral or abdominal pathology. Family history revealed no factor likely to favourise sudden death. The sports involved corresponded with various standard activities in France. Patients aged under 35 had a wide range of cardiac diseases. Almost all those aged over 35 died as a result of coronary diseases. This study shows the relative rarity but also the difficulty of preventing such accidents, in particular in the young individual.

In order to determine the relationship between myocardial bridging and sudden death, the authors analysed retrospectively the macroscopic and histological features of 19 cases of myocardial bridging of the left anterior descending artery (LAD) out of a series of 930 medicolegal autopsy studies. The patients (15 men and 4 women) had an average age of 39.2 years. A potentially lethal cardiac abnormality was found in addition to the myocardial bridge (ischaemic, cardiomyopathy, conduction tissue lesion) in 11 cases; in the other 8 cases, 7 had minor abnormalities and 1 heart was absolutely normal (a 47 year old woman who died while swimming but not of drowning). All hears had fresh, microscopic, ischaemic lesions in the territory of the LAD artery, demonstrated by LIE staining (basic fuschin). The anatomical lesions of the coronary arteries at the site of bridging were varied: 11 dense collagen fibrosis of the adventicia, 16 intimal fibroses of varying degrees of thickness (10 circumferential), 2 atherosclerotic plaques (a 40 and a 54 year old man), 2 recent thromboses (1 at the site of the bridge in a 50 year old man, and the other just distal to the bridge in a 25 year old man). In only 1 case (39 year old woman) there were no microscopic changes of the LAD artery at the site of the myocardial bridge. The hypothesis of the responsibility of the myocardial bridge in the occurrence of sudden death, either during an acceleration of the cardiac rhythm (milking effect) or by thrombotic or spastic phenomena, cannot therefore be excluded.

Cardiologists rarely encounter tumoral pathology, the commonest being the myxoma which presents more often as cardiac rather than neoplastic disease. A few cases of "malignant or metastatic cardiac myxoma" have been reported in the literature. We have not observed this behaviour in the follow-up of 100 cases of myxoma between 1959 and 1989. However, during the same period, 14 intracardiac sarcomas (operated or not) were seen, which were all rapidly fatal. A critical review of the cases of metastatic cardiac myxomas reported in the literature enabled us to classify them into three groups: the first, 9 cases of false myxomas. They were cardiac sarcomas and therefore a histopathological diagnostic error had been made. The second group comprised false metastases. They concerned arterial embolism of fragments of myxoma causing ischemic infarction or infiltration of the arterial wall by the myxoma, damaging the artery and resulting in fusiform aneurysm. This cannot be accepted as a true metastasis: a number of examples are given as they are too many to be named individually. The cardiac and cutaneous myxomas observed in Carney's complex may be included in this category. The third group has to be discussed case by case: these "metastatic myxomas" can all be explained logically and malignancy can be excluded; they are few in number (5 cases!) but often published several times by different authors. In our opinion, a carcinologically "malignant" metastatic myxoma remains a questionable pathological entity. This does not mean that a myxoma cannot be lethal: its intracardiac position may cause fatal valvular obstruction, or it may give rise to fatal embolism.(ABSTRACT TRUNCATED AT 250 WORDS)