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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.

Aims 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. Methods and results 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. Conclusions 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.

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.

We report on two women presenting with cough and fever, 4 and 7 months, respectively, after starting breast radiation therapy following surgery for breast carcinoma. Chest roentgenogram and computed tomographic (CT) scan demonstrated alveolar opacities, initially limited to the pulmonary area next to the irradiated breast, but later migrating within both lungs. Intra-alveolar granulation tissue was found in transbronchial lung biopsies. Corticosteroid treatment resulted in dramatic clinical improvement, together with complete clearing of the pulmonary opacities on chest imaging. However, clinical and imaging relapses occurred when corticosteroids were withdrawn too rapidly; with further improvement when they were reintroduced. The reported cases clearly differ from radiation pneumonitis. They were fairly typical of cryptogenic organizing pneumonitis, also called idiopathic bronchiolitis obliterans organizing pneumonia, with the exception of the radiation therapy, partially affecting the lung, which had been performed within the previous months. Since focal radiation therapy involving the lung may induce diffuse bilateral lymphocytic alveolitis, we hypothesize that this may "prime" the lung to further injury, leading to cryptogenic organizing pneumonitis.

A 39 year old woman with severe primary biliary cirrhosis progressively developed exercise dyspnoea due to airflow obstruction. Sjogren's syndrome was not present. Bronchial and pulmonary biopsies demonstrated constrictive lymphocytic bronchitis/bronchiolitis, possibly a component of a generalized autoimmune process in this patient.

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.

This report concerns the first case of disseminated infection with Scedosporium prolificans (S. inflatum) that occurred in a patient who had received an organ transplant and was verified at autopsy. The patient underwent single (right)-lung transplantation and subsequently died on the 33rd postoperative day in a context of septic shock. S. prolificans was isolated from various specimens, including two cultured blood samples. The organism was associated with Candida albicans in some of these specimens. Autopsy revealed the presence of numerous nodular abscesses in the lungs, myocardium, kidneys, spleen, and gall bladder. These abscesses contained mycelial filaments and numerous oval conidia suggestive of Scedosporium species but no yeasts. MIC studies demonstrated the resistance of the S. prolificans isolate to amphotericin B, fluctyosine, miconazole, ketoconazole, and itraconazole, whereas the isolate of C. albicans was susceptible to amphotericin B, flucytosine, miconazole, and ketoconazole. From the data from our case and six published reports of cases of disseminated S. prolificans infection, it can be concluded that this filamentous fungus is a new agent responsible for the most serious mycoses in both neutropenic patients and patients who have undergone organ transplantation.

Lymphangiomyomatosis (LAM) consists of smooth muscle (SM) cell proliferation of unknown origin involving the lymph nodes and the lung interstitium. From morphological studies showing both SM differentiation of the proliferating cells and lymphatic hyperplasia, hypotheses were suggested concerning the origin of the proliferation. Two cases of LAM were investigated by electron microscopy and immunohistochemistry; tissues were obtained by lymph node and open lung biopsies. Cytoplasmic and matrix protein markers were used in order to clarify the pattern of differentiation of the proliferating cells and to characterize their connective tissue environment. The proliferating cells present ultrastructural characteristics of SM cells; they contain vimentin, desmin, and alpha-SM actin and are devoid of Factor VIII, favouring a parieto-arterial origin. The connective tissue matrix inside the infiltrate is composed of interstitial collagens and basement membrane components. At the late stage of the disease, remodelling of the interstitial matrix accompanies the infiltrate and remains perilesional.

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.

HIV infection can provoke vascular complications, although they are not presently viewed as one of its most serious manifestations

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.

A 47 year old woman developed idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) presenting with haemoptysis and diffuse multiple cavitary nodules. The disease was histologically confirmed by open lung biopsy after other entities had been excluded. The patient responded to a course of corticosteroids. This BOOP should be added to the list of diseases with multiple cavitary nodules.

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.

We report the case of a woman who presented with dyspnoea whilst taking minocycline for acne. Imaging features of bilateral patchy alveolar opacities suggested a diagnosis of bronchiolitis obliterans organizing pneumonia, which was confirmed by lung biopsy. The patient improved, partially, after stopping minocycline, and then completely on treatment with corticosteroids, without relapse when these where stopped 8 weeks later.

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.

Nonspecific interstitial pneumonitis with fibrosis has been individualized within the group of idiopathic diffuse interstitial pneumonias by pathological criteria. It is differentiated from usual interstitial pneumonitis by the temporal uniformity of the lesions, a prominent inflammatory interstitial infiltration, and the absence of honeycombing. Clinical and functional symptoms are those of diffuse interstitial pneumonitis. An etiology may be found in about half the cases, including connective tissue disease, exposure to organic antigens, or recent acute lung injury. Computed tomography of the chest shows bilateral ground glass opacities, and alveolar opacities with a peribronchiolar or patchy distribution. Prognosis is rather good, since a majority of patients improve when treated with corticosteroids or with an association of corticosteroids and immunosuppressive drugs. These etiologic and prognostic features justify the individualization of nonspecific interstitial pneumonitis with fibrosis as a distinct clinicopathological entity.

The overprevalence of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) in women remains unexplained. Microchimerism pathogenicity has been discussed in some systemic diseases. We tested history of pregnancy as a risk factor for GCA.Prospective, multicenter case-control study with multiple, age-matched, control groups. Patients have been included in 40 different centers. The first control group has been randomly selected in the general population, consecutive hospitalized patients in two geographically distant departments of internal medicine made up the second and third ones.Three hundred and fifteen patients (249 GCA and 66 PMR), 242 general population controls, 333 in the first hospitalized control group, and 355 in the second, have been included in the 1991-1998 period. Pregnancy has been constantly protective against GCA/PMR (Wilcoxon rank sum test: P = 0.0001, 0.0005, and 0.054, respectively, for the three control groups), more particularly for parity equal or greater than 4 (OR = 0.32, 95% CI: 0.18-0.57, P = 0.00003; OR = 0.44, 95% CI: 0.26-0.74; P = 0.0009, and OR = 0.42; 95% CI: 0.25-0.71, P = 0.0006, respectively). In multivariate analysis, risk for GCA on pre-existing degenerative, vascular disease is decreased by half for each pregnancy (OR = 0.49, 95% CI = 0.27-0.90, P = 0.022).Contrary to the initial hypothesis, multiparity is a protective factor against GCA. Mechanism is unknown.

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.

From 1978 to 2000, 692 patients underwent a heart transplantation (HT). 137 survived beyond the 10th year. We evaluated morbidity, mortality and immunosuppressive therapy. Population : 103 men and 34 women : Group (G) I : 10-30 y.o.(n515) G II : 30 250 y.o. (n556) GIII : 50 70 y.o. (n566). For those who had survived already 10 years, actuarial survival was 80% at 15 years following HT and 62.5% at 20 years . Mortality slope of 3%/ year was similar to the 0-to-10 year survival curve. 5 women gave birth to 7 children and 4 men had 5 kids, all healthy. Discussion : probability of surviving 20 years following HT is above 60% in this group of 10 year surviving patients. 50 % of the patients transplanted before being 50 y.o. worked after HT and 45% acknowledged no complication. Conclusion : Immunosuppression in HT patients having survived 10 years after their HT is associated with a high rate of complications relative to the low incidence of clinically relevant chronic rejection. Mortality causes (n527)

We report a case of BOOP with severe hypoxemia and orthodeoxia. Chest X-ray showed bilateral infiltrative pneumonia and computed tomography scan revealed consolidations on lower lobes. Diagnosis of right-to-left shunt was made on the increase of alveolar-arterial oxygen difference under FIO2 100%, accented in standing position. Contrast-enhanced echocardiography and technetium-99 macroaggregated albumin body scanning did not show anatomical right-to-left shunt and permitted the diagnosis of intrapulmonary shunt at the capillary level because of intra-alveolar organisation. Treatment with corticosteroids and cyclophosphamide led to clinical, and radiological improvement with correction of the hypoxemia and the shunt.

After 30 years of experience comprising more than 3000 suspension laryngoscopies, we are putting forward a classification of the main benign lesions of the vocal cords. Among the acquired group, we make a distinction between those caused by vocal overuse and abuse and those with a cause within the vocal cord. Congenital lesions are certainly more common than is usually thought. The hypothesis of a congenital origin would certainly justify a larger prospective study.

Percutaneous fine-needle aspiration is a well established method for the diagnosis of peripheral lung lesion. In order to compare different methods of aspiration, we analyze retrospectively two different series: 267 fine needle aspirations (FNA) compared with the histological diagnosis on surgical specimens and 292 lung biopsies using a coaxial technique with comparison between cytological diagnosis--smears and imprints--and histological diagnosis simultaneously obtained on the same specimen. The sensitivity (91%), the specificity (90%) and the overall typing accuracy related to the histological types obtained by FNAB are equivalent to those of the literature. The low rate of pneumothorax in the series (6%) is related to the use of immediate interpretation of the specimen. Automated biopsy with a coaxial cutting needle provide cytological specimens--smears and imprint--with a high rate of sensibility (95.3%) and of sensibility (98%). The overall sensitivity of the cytological methods alone is better than biopsy (95.3% vs. 92.9%), but the typing accuracy is not as good as biopsy alone (98% vs. 100%). False-positive and false-negative diagnoses are the same in both series. In conclusion the percutaneous aspiration method choose to establish a morphological diagnosis in lung lesion depends now on the habits of the radiologist and the pathologist.

Sarcoma of the common pulmonary artery is a rare malignant tumor that can mimic pulmonary embolism.We report a case of a pulmonary artery sarcoma that occurred in a 60-year-old woman and had an unusual (4-year duration) evolution.Early diagnosis with adequate surgical procedures (complete resection) leads to better prognosis.

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

Epicardial radiofrequency catheter ablation of the atria in the open-chest dog has been shown to reduce inducibility of atrial fibrillation. Video-assisted endoscopic techniques decrease the operative trauma in adult thoracic surgery. We report our results of video-assisted thoracoscopic radiofrequency catheter ablation of the atria for the prevention of atrial fibrillation induction in canines. In 12 consecutive anesthetized dogs, induction of sustained atrial fibrillation was reproducibly obtained by burst pacing and cervical vagal stimulation. In six dogs, biatrial ablation was performed through right and left minithoracotomies and guided by video-assisted endoscopic techniques. The remaining six dogs underwent a video-guided left atrial procedure. Long continuous and transmural lesions were produced using epicardial temperature controlled radiofrequency energy delivered according to a simplified maze approach. Transmural lesions were demonstrated at the end of the study by examination of the heart. Sustained atrial fibrillation was still inducible after the right atrial ablation but sustained atrial fibrillation could not be induced following left atrial ablation. In acute canine studies: (1) epicardial radiofrequency catheter ablation of the atria is feasible using video-assisted endoscopic techniques; (2) ablation extended or confined to the left atrium appears to be effective in preventing the inducibility of sustained vagal atrial fibrillation; and (3) ablation of the right atrium alone had no antiarrhythmic effect.

The existence of a bronchial foreign body is an unusual cause of haemoptysis. We observed a sixty two year-old women who presented several medium-abundance haemoptysis. They were associated with a systematic alveolar-interstitial radiological picture of the ventral upper right lobe. A right upper lobectomy showed that an old bronchial foreign body (piece of bone) was responsible for the systematic intra-alveolar bleeding. Though most of the breathed foreign bodies are expressed into immediate symptoms, some of them remained undiagnosed and may be responsible for haemoptysis, infectious complications, atelectasis and for bronchiectasis. Their extraction through endoscopy or most often surgery is necessary for a proper recovery. In spite of histopathological differences between foreign bodies, broncholithiasis and lung tumor the diagnosis may be difficult clinically and on radiology.

OBJECTIVE To assess the potential role of allo-immunization, either by former pregnancies, or by a history of blood transfusion, in the pathogenesis of giant cell arteritis and polymyalgia rheumatica. METHODS Two hundred and eighty-five incident female cases and 186 age-matched, population-based female controls were prospectively included in a multicentre case-control study. RESULTS The number of pregnancies was significantly lower in cases than in controls (nulliparous: 21.55% vs 12.90%; > or =4 pregnancies: 16.25% vs 27.42%; Wilcoxon rank sum test: P = 0.0019) in biopsy-proven or negative temporal arteritis and, to a lesser extent, in polymyalgia rheumatica. No difference was found for history of blood transfusion. Pregnancies remained negatively associated with the disease in a multivariate analysis including cardiovascular risk factors such as smoking or a pre-existing peripheral vascular disease. CONCLUSION Former pregnancies are not a risk factor for giant cell arteritis. Pregnancies may be protective thanks to an effect of the associated hyperoestrogenic state against alterations of the artery wall, as suggested in animal models.

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.

Papillary fibroelastoma is a seldom reported tumour. It usually occurs in adults and develops on the aortic and mitral valves. It is not different of giant Lambl excrescences and differential diagnostic can be difficult with the myxoma. Its systematic surgical ablation is justified by the important risk of embolic complications. It has rarely been reported in children. We report a case peculiar by fortuitous diagnostic, tricuspid site, large size and occurrence in a 3-year old child.

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.