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Four cases of lymphomatoid granulomatosis are reported, three of them involving the lung. Histological features included a true angiocentric and angiodestructive polymorphic cellular proliferation. This included histiocytes, plasma cells, many reactive T-cells and rare large, atypical cells which were of the B phenotype. Epstein-Barr virus was detected in the atypical cells by in situ hybridization in three cases, with expression of both latent membrane proteins (LMP)-1 and Epstein-Barr nuclear antigen-2 in two cases and expression of only LMP-1 in the third case. Expression of both of these proteins suggests a defect in the T-cell-mediated immunity and that Epstein-Barr virus is not only a silent passenger but may also be involved in the pathogenesis of the disease. This could have implications for therapy.

To evaluate bronchiectasis detected with thin-section computed tomography (CT) as a potential early finding of chronic lung allograft rejection.Forty patients were studied at least 6 months after transplantation. Follow-up CT scans were obtained 6-48 months after transplantation and were retrospectively reviewed for evidence and anatomic extent of bronchiectasis. Irreversible dysfunction of the lung transplant, called the bronchiolitis obliterans syndrome (BOS), was staged according to pulmonary function testing. Pathologic proof of bronchiolitis obliterans was obtained.BOS occurred in 14 (35%) of the 40 patients. When considering bronchiectasis preceding BOS, bronchiectasis was a predictor of chronic rejection with a sensitivity of 14%, a specificity of 77%, a positive predictive value of 25%, and a negative predictive value of 63%. Bronchiectasis appeared concomitantly with BOS in eight (67%) of 12 patients.The diagnosis of chronic rejection relies on the degree of impairment of pulmonary function. When the diagnosis is only suspected on the basis of minimal impairment, CT can aid in the diagnosis of rejection by depicting early pulmonary changes.

Over the period 1983-1990, 10 cases of infective native-valve endocarditis as a result of Chlamydia were seen. All patients were men, with a mean age of 42 years, and none had a history of exposure to Chlamydia psittaci. Symptoms, such as weight loss and anorexia, with fever in eight cases, had persisted for at least 2 months before admission. Haemodynamic failure was present in seven patients, and neurological signs in four. The aortic valve was involved in seven cases, the mitral valve in one and both valves in two. Vegetations, often fingerlike, were observed by echocardiography in nine cases. All patients required valve replacement, and three died in the year following diagnosis. Blood cultures were consistently negative in all cases, and no antibiotics had been given before admission. Significant titres of complement fixing anti-chlamydial antibodies were present in six cases, and micro-immunofluorescence using the three chlamydial species showed cross-reacting antibodies in all nine cases studied, with transient IgM positivity in six cases. Histologically, the leaflets were fibrosed and infiltrated by large macrophages containing dark red granules after Machiavello staining. These granules were specifically stained by immunofluorescence using monoclonal antibody to Chlamydia common antigen, but not by that specific for C. pneumoniae. No organisms were seen after Gram staining, and no positive chlamydial immunofluorescence was seen on sections of valves from patients with staphylococcal or streptococcal endocarditis.

Giant cell arteritis is the most frequent vasculitis. Cardiovascular events such as cerebrovascular accident or ischemic heart disease may occur in patients with giant cell arteritis. However, their real incidence, as well as their relative risk compared to the general population, remains unknown.To assess in a prospective, double cohort study, the incidence of cardiovascular events in giant cell arteritis patients compared to controls, after controlling for cardiovascular risk factors.We included on predefined criteria 432 newly diagnosed patients with giant cell arteritis, each assigned to sex- and age-matched controls randomly selected from the general population. Cardiovascular risk factors (high-blood pressure, diabetes, smoking, hypercholesterolemia and preexisting peripheral vascular disease) were collected at inclusion. During the 24-month follow-up, all cardiovascular events were collected. After stratification for cardiovascular risk factors, a log-rank test was performed to compare cases and controls. A parametric survival model was used for multivariate analysis.Cardiovascular events all combined were significantly increased in patients with giant cell arteritis (RR = 2.15 [1.21-3.81], P = 0.009), and were mainly associated with age (P = 0.0001), past history of cardiovascular disease (P = 0.023) but also with giant cell arteritis (P = 0.009). However, each subset of cerebrovascular accident (RR = 2.42 [0.84-7]) or ischemic heart disease (RR = 1.67 [0.72-3.89]) increased but did not significantly.Cardiovascular events incidence is increased in patients with giant cell arteritis, and prescription of preventive antiagregant treatment may be discussed.

We report a patient with a papillary fibroelastoma arising from the left ventricular posterior wall. The tumor was detected incidentally during echocardiography undertaken to evaluate aortic stenosis. Possible complication from tumor embolization was avoided by surgical resection during aortic valve replacement.

Background— Sudden death is a possible consequence of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Prevalence of ARVC/D in unexpected sudden cardiac death (USCD), however, remains imprecise, as do circumstances of death and ARVC/D-associated gross and microscopic findings, especially His bundle anomalies. Methods and Results— We reviewed 14 000 forensic autopsies required by judicial authorities from January 1980 to January 1999 in a 2 000 000-resident area. Age, gender, and circumstances of death were recorded. Hearts were examined macroscopically and microscopically. In this series, the ARVC/D group accounted for 200 consecutive cases (10.4%) of USCD, including 108 males and 92 females (average age 32.5 and 34.5 years, respectively). Nearly one third of deaths occurred during the fourth decade of life. Circumstances of death were various, but 75.6% occurred during everyday life events (at home, 63.1%; in the street, 6.6%; or at work, 6.1%); only 7 cases (3.5%) occurred during sports activity. Nineteen cases (9.5%) happened during the perioperative period. Adipose infiltration of the right ventricle was either isolated (20%) or associated with fibrosis (74.5%) and lymphocytes (5.5%). A total of 14.5% of cases had cardiac hypertrophy, assessed by an increase in heart weight and/or left ventricular wall thickness. In most cases, the His bundle and its branches were abnormal either because of infiltration of adipose tissue (8.1%), fibrosis (54.3%), or both (5.6%). Conclusions— In ARVC/D, both sexes are equally affected, and there is a peak of risk during the fourth decade. Death most frequently occurs during sedentary activity. His abnormalities and left ventricular hypertrophy may be associated with ARVC/D.

We carried out a retrospective analysis of 1500 forensic autopsies following sudden unexpected cardiac death. This analysis showed a group of 43 cases that could have been related to surgery and/or anaesthesia. Pathological examination revealed the existence of cardiac lesions in 40 cases: arrhythmogenic right ventricular cardiomyopathy (14 cases), coronary artery disease (9 cases), cardiomyopathy (8 cases), structural abnormalities of the His bundle (7 cases), mitral valve prolapse (1 case) and acute myocarditis (1 case). These disorders are compatible with a paroxysmal (rhythmic) phenomenon at the origin of the cardiac arrest. Identification of the cause of death in a patient who was presumed to be at low risk may provide major relief to the patient's family and the medical staff.

A 59 year-old man with a history of asbestos exposure presented with a right pleural effusion and a diffuse pleural thickening with focal calcifications on chest X-ray. Cytological examination of pleural fluid indicated malignant mesothelioma. A biopsy specimen showed malignant mesothelioma surrounding a fragment of mature bone. The patient was treated with intrapleural interferon, but relapsed 3 years later. A fresh biopsy specimen showed round tumor cells surrounding osteoid substance. Only ten cases of this rare variant of malignant mesothelioma with osteoblastic heterologous elements have been reported in the literature. The most difficult differential diagnosis is primary pleural osteosarcoma.

The retrospective analysis of 1700 forensic autopsies over 17 years (1981-97) following unexpected sudden cardiac death revealed a group of 50 cases that could have been related to surgery and/or anaesthesia. Patients were young with no history of cardiac disease. Surgery was performed for uncomplicated disorders, all classified as ASA 1. Cardiac arrest took place at induction of anaesthesia in 16% of cases, during surgery in 64% and at the end of surgery in 20%. Investigation and expertise reports ordered by the public prosecutor revealed none of the typical causes of death usually associated with surgery or anaesthesia. Pathological examination showed cardiac lesions in 47 cases: arrhythmogenic right ventricular cardiomyopathy in 18 cases, coronary artery disease in 10 cases, cardiomyopathy in eight cases, structural abnormalities of the His bundle in nine cases, mitral valve prolapse in one case, and acute myocarditis in one case. Identification of the cause of death of patients at low risk may provide major relief to the family of the patient and the medical staff.

An 18-year-old cyclist was referred to the laboratory for unexplained pain in his left lower limb during exercise. He had started competition at 14 years of age and had cycled ≈10 000 km/y in the preceding 5 years. He had complained for 18 months of a subjective sensation of a painful and swollen left thigh at maximal exercise. Different …

Aggressive primary mediastinal non-Hodgkin's lymphomas (NHL) represent a particular entity among intrathoracic neoplasms. Twenty-nine patients with primary mediastinal aggressive NHL diagnosed and treated in the author's institution were studied. According to the Revised European-American Lymphoma (REAL) classification, there were 15 diffuse large B-cell, eight T-lymphoblastic, four anaplastic, one large T-cell and one Burkitt's lymphomas. The study group consisted of 14 females and 15 males, with a mean age of 38 yrs. Symptoms arose from an aggressive anterior mediastinal mass, with a high prevalence of superior vena caval syndrome, pleural, and pericardial effusions. At the time of diagnosis, disease was confined to supradiaphragmatic areas in 24 patients, while subdiaphragmatic nodal or extranodal involvement was also present in five. All patients received a combination of aggressive chemotherapy regimens, mainly according to the French protocols for the treatment of NHL. A chest radiograph response of

The retrospective analysis of 1500 forensic autopsies after sudden cardiac death showed that 80 (77 men, three women) had died following sport, for which they had been inadequately trained. The chosen sport (both dynamic and static), and the cardiac pathology discovered during autopsy make it possible to divide the population into two groups. Group 1 were those under 30 years of age (27 cases) engaged in jogging, gymnastics, rugby, tennis and boxing who suffered from hypertrophic cardiomyopathy (29.6%), arrhythmogenic right ventricular cardiomyopathy (25.9%), non-atherosclerotic (14. 8%), aortic stenosis (7.4%), atrial septal defect (3.7%), stenosing coronary atherosclerosis (3.7%), and structural abnormalities of the His bundle (3.7%). Group 2 were those over 30 years of age (53 cases), engaged in swimming, cycling, jogging and football. The cardiac lesions responsible were stenosing atherosclerotic coronary disease (49%), non-atherosclerotic coronary disease (1.8%), hypertrophic cardiomyopathy (20%), obstructive cardiomyopathy (4.8%), structural abnormalities of the His bundle (7.4%), myocardic bruise scar (4%), and arrhythmogenic right ventricular cardiomyopathy (3. 7%). In both groups, dilated cardiomyopathy occurred with identical frequency (11%).Conclusions The lesions discovered are the same as those identified in professional athletes, when the body tries to avoid mortal rhythmic decompensation in the case of an over-loading volume and tension during an ill-adapted effort. Forensic autopsy should establish these anomalies because the transmissible genetic characteristics of some of them could underline the need for check-ups in other members of the family.

Primary cardiac sarcomas are rare and aggressive tumours. The aims of this study were to precisely classify cardiac sarcomas according to their pathology, and to determine their clinicopathological features and prognosis.Twenty-four primary cardiac sarcomas were studied. Clinical features and follow-up of all patients were collected. Histological diagnoses were obtained by combining both morphological features as described in soft tissue counterparts and the immunoprofile of the tumours. The 24 cases were classified as undifferentiated sarcoma (nine cases), angiosarcoma (six cases all located in the right atrium), leiomyosarcoma (six cases), malignant fibrous histiocytoma (one pleomorphic and one giant cell type) and synovial sarcoma (one case). Patients included 14 men and 10 women, with a mean age of 46 years. Clinical manifestations were protean, often delaying the diagnosis. Dyspnoea resulting from obstruction of the cardiac chambers was the most common symptom. Echocardiography and magnetic resonance imaging were useful respectively to detect and to evaluate tumour extension. Complete macroscopic resection was possible in only 33% of patients. The most common cause of death was local recurrence of the tumours (50%), even in the cases of complete macroscopic resection. Whatever the treatment, the prognosis was poor with a mean survival of 16.5 months after diagnosis.All types of sarcomas may be observed in the heart with a predominance of undifferentiated sarcomas. Histological grading, unlike histological type, seems to correlate with survival which remains extremely poor.

After a period during which cardiac papillary fibroelastomas were considered incidental autopsy findings cerebral and coronary arteries embolism proved their aggressiveness. Echocardiography is now able to identify them and surgical resection is rapidly required. Sea anemone like macroscopic pattern is characteristic with finely villous masses, each frond being at microscopical examination formed by a central fibroelastic core surrounded by a myxomatous layer overlied by endothelial cells. Histogenesis remains elusive and we tried to clarify it by immunohistochemical analysis of 8 of the 20 cases studied (10 autopsies, 10 surgical resections). Morphological and immunohistochemical data show that endothelial cells play the most important part in abnormal formations.

Although suspected, a viral etiology has never been proven in giant cell arteritis (GCA). We tested for viruses known to induce multinucleated giant cells in human pathology, which include the parainfluenza viruses (HPIV), respiratory syncytial virus, measles virus, herpesviruses type 1 and 2, and the Epstein-Barr virus.A multicenter case-control study on incident cases of temporal arteritis (TA) and polymyalgia rheumatica (PMR). Population based age and sex matched controls were randomly selected. Serological tests for IgG and IgM directed against the viruses listed above were performed, on blood samples taken at the time of clinical diagnosis.Three hundred five new patients were included over a 5 year period, of whom 159 presented with positive biopsy TA, 70 with negative biopsy TA, and 76 with negative biopsy PMR. Thirty-eight percent of cases versus 20.9% of controls were positive for IgM directed against HPIV (p = 0.00005). The association was stronger in the positive TA subgroup [positivity rate 43.31%; odds ratio with controls 2.89 (95% CI 1.82-4.60, p = 0.000006)] than in the PMR or negative biopsy TA subgroups. Only HPIV type 1 was associated with the disease, regardless of the season or the geographical origin of the cases. Positivity rates for HPIV types 2 and 3 and for the other viruses tested were similar in cases and controls.Our findings suggest that reinfection with HPIV type 1 is associated with the onset of GCA in a subset of patients, particularly in cases with positive TA biopsy.

To assess the potential pathogenetic role of cardiovascular risk factors measured at diagnosis in temporal arteritis.Four hundred newly diagnosed patients and their age- and sex-matched controls randomly selected from the general population were included in a prospective multicentric cas-control study.In female patients, smoking increased the disease risk by a factor of 6 (95% CI: 2-17, p = 0.00006), and by a factor of 17 in case of10 pack-years. Pre-existing peripheral arterial disease increased the risk by a 4.5-fold factor (95% CI: 2-11, p = 0.0003), both in patients with a positive and those with a negative biopsy. Smoking alone appeared as a risk factor for polymyalgia rheumatica (OR: 3,64, 95% CI: 1.07-12.40). In male patients, none of the cardiovascular risk factors studied was positivity associated with the disease.Smoking and a pre-existing peripheral arterial disease are independently associated with temporal arteritis in women. Risk factors for disease with an imbalanced sex ratio should be studied separately for each sex in matched case-control studies in order to avoid matching bias.

Background Native valve strands might be related to the acute stage of thrombosis or might suggest a long-term valvular change. We aimed to estimate changes in the strands in patients with stroke through a serial transesophageal echocardiographic (TEE) study. Methods and Results A study was conducted among patients who were referred for TEE for stroke or cardiac pathology. Patients had TEE examinations with a 5-MHz multiplane TEE probe. Echocardiography was repeated 3 months later in patients with stroke. TEE was performed in 180 patients admitted to cardiology units and in 160 patients referred to neurology units. Among 34 patients with valvular strands, 30 were referred to neurology for stroke, whereas 4 patients were admitted to cardiology (18.8% versus 2.2%, difference 16.5%, 95% confidence interval 10% to 22.9%, P = .001). Strands were located on the mitral valve in 16 patients, the aortic valve in 6 patients, and both left heart valves in 8 patients. Among the 38 valves with strands, 17 (44.7%) were morphologically normal, 4 (10.5%) were thickened, 7 (18.4%) were redundant, and 10 (26.3%) had both abnormalities. TEE showed other abnormalities in 16 (53.3%) patients, whereas 14 patients had only strands. Twenty-six (86.6%) patients had a second TEE study 3 months later. Strands were not found in 4 (15.4%) patients (95% confidence interval 4.3% to 34.9%). Conclusions Valvular thickening or redundancy may predispose valves to strand formation. Native valve strands usually persist and thus reflect a chronic valvular change. (Am Heart J 1998;136:1065-9.)

To assess the role of cardiovascular risk factors, measured at the time of diagnosis, in the pathogenesis of giant cell (temporal) arteritis (GCA).Four hundred new patients with GCA or polymyalgia rheumatica (PMR) and population-based, individually age- and sex-matched controls were included in this multicenter, prospective case-control study. Each participant was evaluated by review of the medical history and by clinical and laboratory assessments.Among women, smoking was associated with a 6-fold increase in risk (P = 0.00006, 95% confidence interval [95% CI] 2-17), heavy smoking with a 17-fold increase in risk, and previous atheromatous disease with a 4.5-fold increase in risk (P = 0.0003, 95% CI 2-11) of GCA in both the biopsy-positive and biopsy-negative GCA groups; only smoking appeared to be a risk factor for PMR in women (odds ratio 3.64, 95% CI 1.07-12.40). Among men, no risk factor was found to be significant.Smoking and previous arterial disease were independently associated with GCA in women. In order to avoid matching bias, risk factors for diseases with an unbalanced sex distribution should be studied separately in each sex, using a sex-matched, case-control study design.

The growing number of reports of surgery for papillary fibroelastomas of the heart led us to evaluate the diagnostic potential of ultrasonography in patients with cerebral or coronary signs and to assess the efficacy of anticoagulant therapy in preventing recurrent cerebral ischemia and disease progression after resection.Ten cases of echographically diagnosed fibroelastoma of the heart treated by surgery were analyzed together with cases reported in the literature.Transesophageal echography has been shown to be the superior method. Surgical resection has given good results and the postoperative course is always excellent. Recurrent embolism occurred in two of our cases despite well-conducted anticoagulation.Surgical resection should be performed as early as possible because anticoagulation does not appear to sufficiently protect against embolic events, particularly cerebral events.

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.

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.

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.

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.

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.

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.

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.

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 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)

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.

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 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 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)