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[Chronic inflammatory bowel disease and cardiovascular complications].

The most common extraintestinal manifestations of Crohn's disease and ulcerative colitis are iritis and uveitis, primary sclerosing cholangitis (PSC) and nodal erythema and pyoderma gangrenosum. Complications within the cardiovascular system seem to be uncommon, but there are no systematic investigations concerning the epidemiology of these manifestations. There are more than 100 cases reported about pericarditis and perimyocarditis in patients with inflammatory bowel disease. Other patients with Crohn's disease or ulcerative colitis suffer from vasculitis, representing a further mechanism of inflammatory diseases of the cardiovascular system. There are several case reports showing a combination of Takayasu's arteritis and Crohn's disease, and cross-reacting antibodies against gut mucosa and aortic tissue were found. Some patients developed thrombotic complications by activating the coagulation system, which can result in atrial thrombi, embolism of the pulmonary arteries, myocardial infarction and disseminated intravascular coagulopathy (DIC). Furthermore, a few case were reported about atrio ventricular blocks, amyloidosis of the heart, dilative cardiomyopathy and endomyocardial fibrosis in patients with chronic inflammatory bowel disease. Here, a 27-year-old patient with known ulcerative colitis for 2 years is reported, who presented in the authors' department with unstable angina pectoris. Coronary angiographic examination was immediately performed and diffuse intracoronary thrombi were found, which could be removed by the catheter procedure. A myocardial infarction did not develop. Because of positive anti neutrophil cytoplasmic antibodies (p-ANCA) a p-ANCA-positive arteritis of the coronary vessels with intracoronary thromboembolism due to ulcerative colitis was diagnosed. Systematic studies or investigations concerning the epidemiology of the cardiovascular complications are still lacking, so that an overview about the published data is given.

Adult↗

[Restrictive cardiomyopathy of the right ventricle: differential diagnosis. Series of three cases].

The cardiomyopathies are defined as a primary disease of the myocardium and occur in children as well as in adults. Restrictive cardiomyopathy is the least common type of cardiomyopathy and is characterized by the restriction of ventricular filling. This condition can affect one or both ventricles. The appropriate diagnostic tool includes a detailed clinical record supported by imaging and hemodynamic studies. The purpose of this study was to evaluate adult patients with suspicion of restrictive miocardiopathy of the right ventricle, from the out-patients clinic using echocardiogram, magnetic resonance imaging, and hemodynamic studies and to establish a differential diagnosis with other cardiovascular diseases. Between May 2003 to January 2006 three patients with different initial diagnoses, such as Ebstein's anomaly, interatrial septal defect, and tumor of the right ventricle were studied. However, once the protocol of the study was completed, the final diagnosis was of right ventricular restrictive cardiomyopathy by endomyocardial fibrosis.

Adult↗

[A case of cardiomyopathy due to allergic bronchopulmonary aspergillosis].

We report a rare occurrence of cardiomyopathy associated with allergic bronchopulmonary aspergillosis (ABPA). A 49-year-old man with a history of bronchial asthma was referred to the Matsuyama Red Cross Hospital for evaluation of the abnormal shadow on his chest X-ray. Laboratory examination showed blood eosinophilia and marked elevation of serum IgE concentration and IgE antibody to A fumigatus. The immediate and late skin reactivities to A fumigatus antigen were both positive. The diagnosis of ABPA was made. Treatment using prednisolone was effective in ameliorating the symptoms. However, he was admitted again due to dyspnea, edema and anorexia 6 months later. Chest X-ray, ECG, UCG and scintigraphy suggested severe cardiac failure. The clinical diagnosis of hypertrophic cardiomyopathy, and the pathohistological diagnosis of endomyocardial fibrosis were made by cardiac catheterization and biopsy of endocardium. Retrospectively, cardiomegaly had gradually increased during the past several months while peripheral blood eosinophilia had continued. All these data strongly suggested that eosinophilia due to ABPA might cause severe cardiac damage.

Aspergillosis, Allergic Bronchopulmonary↗

Valve extension with glutaraldehyde-preserved autologous pericardium. Results in mitral valve repair.

Preliminary experimental studies in our laboratory have shown that autologous pericardium treated with glutaraldehyde prevents late deterioration and calcification of the tissue. For this reason, glutaraldehyde-treated autologous pericardium has been used in a series of 64 patients who underwent operations for leaflet extension of the mitral valve between 1980 and 1989. Ages ranged from 2.5 to 60 years (mean 19 +/- 15). The causes of mitral valve insufficiency were rheumatic fever (69%), bacterial endocarditis (17%), congenital (8%), endomyocardial fibrosis (4.5%), and trauma (1.5%). The autologous tissue was fixed in a 0.62% glutaraldehyde solution for 15 minutes and rinsed in saline for an additional 15 minutes. Patching techniques varied depending on the site and the extent of the lesion. Associated mitral valve repair techniques (Carpentier's techniques) were mandatory in all patients. The period of follow-up extended from 6 months to 9 years (mean 3.1 +/- 2.5 years). There were no operative deaths in this series, and there was one late death (2%). In the six patients (12%) who underwent reoperation, there has been no case of calcification of the pericardial patch. Postoperative mitral valve function was assessed by bidimensional color Doppler echocardiographic techniques. Mitral valve insufficiency was trivial or absent in 80% of the patients. This experience permits us to conclude that leaflet extension is a simple and safe technique in valve reconstruction, allowing repair of mitral valves that otherwise would need to be replaced. It permits use of an adult-size prosthetic ring in children. Glutaraldehyde-treated autologous pericardium is the material of choice for this type of repair.

Adolescent↗

[Cardiac trichinosis--echocardiographic study].

Trichinosis is an intestinal and tissue infection of man caused by Trichinella spiralis. The most frequent and serious complication is myocarditis. M-mode and two-dimensional echocardiographic studies were carried out on 16 patients with clinical manifestation of trichinosis. The parameters of the left ventricular function, measured by echocardiography were examined, and the results were compared with the same parameters in 50 normal individuals. In 11 patients a variety of morphologic findings, like pericardial effusion, adhesions, focal endomyocardial fibrosis, dilated left ventricular cavity, were found. In one patient intra cavitary thrombus was bound. Pathologic parameters of the left ventricular function were registered in 15 patients.

Cardiomyopathies↗

Metabolic, functional, and histologic characterization of the heterotopically transplanted rat heart when used as a model for the study of long-term recovery from global ischemia.

The objective of this study was to assess the utility of the heterotopically transplanted rat heart as a model in which to assess long-term (0 to 7 days) postischemic recovery. This was achieved by characterizing the metabolic, functional, and histologic changes that occur during the first week after implantation. In the first series of studies (n = 6/group), hearts were subjected to 1 hour of global ischemia at room temperature (21 degrees +/- 1 degree C), during which time they were transplanted into the abdomens of the recipients. A permanently implanted intraventricular balloon was used to measure pressure-volume relationships in the implanted heart 1, 4, 8, 12, and 18 hours and 1, 2, 3, 4, 5, 6, and 7 days after transplantation. Left ventricular developed pressure (70 microliters loading volume) was 125 +/- 14 mm Hg (mean +/- SEM) after 1 hour of reperfusion, declining to 79 +/- 15 mm Hg after 4 hours before increasing to a maximum (158 +/- 14 mm Hg) at 24 hours. The pressure subsequently declined to 108 +/- 16 mm Hg by 3 days, and then remained essentially unchanged for the following 4 days. Heart rate declined to 197 +/- 26 beats/min after 1 hour of reperfusion, recovered to 380 +/- 21 beats/min after 24 hours, and remained above 300 beats/min for the remainder of the experiment. Left ventricular end-diastolic pressure increased progressively to very high levels through the 7-day period (176 +/- 23 mm Hg at 7 days). Analysis of creatine phosphate (CP) and of adenosine triphosphate (ATP) and its breakdown products indicated a loss of high-energy phosphates after 1 hour of ischemia (CP = 4.8 +/- 0.3 mumol/gm dry wt), a recovery to preischemic values after 24 hours (CP = 24.4 +/- 2.5 vs 23.8 +/- 1.3 mumol/gm dry weight in fresh control hearts), and a subsequent decline over the ensuing 6 days (CP = 8.1 +/- 1.5 mumol/gm dry wt at 7 days). In additional studies to assess the functional capacity of the unloaded transplanted heart, hearts were excised 1 hour and 1 day after transplantation and perfused as isolated working preparations: their function was then compared with that of fresh nontransplanted hearts. A time-dependent deterioration of all indices of cardiac function was observed. Morphologic studies of transplanted hearts with and without an inserted and inflated intraventricular balloon revealed a rapid reduction of left ventricular cavity volume during the first 24 hours in hearts without a balloon, and progressive severe fibrosis, endomyocardial necrosis, and inflammation over the 7-day period in hearts in which the balloon was intermittently inflated for functional assessment.(ABSTRACT TRUNCATED AT 400 WORDS)

Abdomen↗

[Echocardiographic manifestations in patients with hypereosinophilia].

In order to assess the degree and type of cardiac involvement in patients with sustained hypereosinophilia, we studied by two-dimensional, M-mode echocardiography and Doppler (4 cases) 20 patients distributed into 2 groups. Group I: 10 patients with the idiopathic hypereosinophilic syndrome. Group II: 10 patients with secondary hypereosinophilia. In group I, 6 patients (60%) had echocardiographic abnormalities consistent with the endomyocardial disease: four apical obliteration of right ventricle, three apical obliteration of left ventricle, three endocardial thickening of the left ventricle, three endocardial thickening of the left ventricle posterior wall, one endocardial thickening of the right ventricle free wall, three subvalvular mitral thickening, three subvalvular tricuspid thickening, two pericardial effusion and two protodiastolic septal notch. These corresponded to 2 cases of endomyocardial fibrosis and restriction, as shown by pathological and hemodynamic study. In only 1 patient from group II echocardiographic abnormalities consistent with right apical occupation and tricuspid subvalvular thickening, with mild regurgitation detected by Doppler, were found. It was concluded that echocardiographic abnormalities are common in patients with idiopathic hypereosinophilic syndrome, even in the absence of clinical features. The development of echocardiographic abnormalities in patients with sustained secondary hypereosinophilia is exceptional and is probably related to duration of eosinophilia. Therefore, we think that echo-Doppler is a fundamental investigation for the diagnosis and follow-up of these patients.

Adult↗

Mediastinal hydatid disease: report of three cases.

We report three patients who had mediastinal echinococcosis and then review the literature. The first patient had a primary anterior mediastinal echinococcal cyst, the second a mediastinal hydatid cyst, secondarily involving the mediastinum and mimicking Hodgkin's disease, and the third had a primary cardiac and pericardial hydatid cyst. In two patients computed tomography (CT) was helpful in making a preoperative diagnosis before any complication could arise. In the third CT was not done and the diagnosis of cardiac echinococcosis at surgery was a surprise. Two large echinococcal cysts compressing the ventricles, which on echography and cardioangiography had the appearance of endomyocardial fibrosis, were removed by total excision. The disease in each patient was correlated with clinical and radiologic findings and was confirmed by tissue microscopy.

Adolescent↗

Eosinophilic myocarditis in CBA/J mice infected with Toxocara canis.

In humans, chronic eosinophilia has been associated clinically with endomyocardial fibrosis and myocardial damage. Mice infected with Toxocara canis have a marked eosinophilia, and develop eosinophil-rich granulomatous lesions in the soft tissues of the body, especially the lungs, liver, brain, and skeletal muscle. Few reports have described myocardial lesions associated with T. canis infections in mice. We examined the hearts of CBA/J mice killed at weekly intervals over an 8-week period for evidence of myocardial damage that might be attributable to eosinophils. Total white blood cell counts and eosinophil counts were obtained during this period, and revealed a peak white blood cell count of approximately 28,000 cells/mm3 at day 7 after infection and a peak eosinophil count of approximately 4,000 cells/mm3 at day 14 after infection. Myocardial lesions in the ventricular wall began as focal infiltrates of eosinophils and histiocytes, then progressed into granulomata containing necrotic debris. Collagen deposition was noted by day 21 after infection. By day 42 after infection, the lesions had contracted greatly because of a loss of cellularity, and consisted mainly of fibroblasts and hemosiderin-laden macrophages. Myocyte damage, characterized by increased eosinophilia and necrosis, was observed. T. canis-infected CBA/J mice thus offer a useful model to study eosinophil-dependent myocardial damage.

Animals↗

The role of endomyocardial biopsy in the diagnosis and treatment of heart disease.

Endomyocardial biopsies (EMBs) have recently become a routine examination method at a number cardiological departments. In general, EMB is indicated whenever it appears necessary to establish or supplement a clinical diagnosis pertaining to a myocardial lesion. Good results can be expected in diffuse processes showing endomyocardial predilection, such as acute rejection and cardiotoxicity or in well-circumscribed lesions such as asymmetric hypertrophy, Löffler's endocarditis or endomyocardial fibrosis. The bioptic confirmation is an auxiliary and positive contribution. The present publication has been based on personal experience with a collection of 550 EMBs. Indications for obtaining EMBs included acute rejection in the cardiac allograft, anthracycline-induced cardiotoxicity, myocarditis, cardiomyopathies, specific heart diseases, idiopathic chest pain and arrhythmias, as well as the differential diagnosis of restrictive versus constrictive heart diseases. The treatise deals with the usefulness and significance of EMB, critical remarks on the problems of EMBs, methodology and evaluation of EMBs and their pitfalls. The above-mentioned indications have been presented as separate chapters comprising the basic problems, discussion of pertinent data from the world literature and correlations between our EMBs with the results published. As a matter of fact, some chapters have been documented by small numbers of personal EMBs (cardiotoxicity, specific heart diseases), but they have been included for the sake of completeness. In the future EMBs appear to remain a useful diagnostic as well as research tool in problems of cardiovascular diseases. An exact interpretation of EMBs requires an experienced pathologist capable of arriving at adequate conclusions from the changes disclosed in EMBs.

Adolescent↗

[Heart involvement in hypereosinophilia: the restrictive or the dilated type?].

As many as 60 patients with hypereosinophilia of different genesis were examined. 40 of them manifested the clinical (congestive heart failure in 6, tachycardia in 10, cardialgias in 3, murmur in the heart in 13, pericardial murmur in 3) and/or ECG signs (disorders of repolarization in 25, His bundle block in 4, rhythm disorders in 7, pathologic Q waves in 2) of heart injury. In 15 out of the 20 examined, the echocardiography data corresponded to the dilated pattern of heart injury despite the fact that the changes in contractile function of the left ventricle were less appreciable than in 28 patients with dilated cardiomyopathy. In 2 patients who died (one from congestive heart failure and one from anaphylactic shock) the presence of endomyocardial fibrosis with heart dilatation was confirmed on postmortem examination. One female patient with right ventricular failure manifested the characteristic signs of isolated injury to the right ventricle. In 3 patients, the endocardium appeared thickened. None of the cases showed the signs of restrictive heart injury. The data obtained indicate that hypereosinophilia may be one of the etiologic factors of dilated cardiomyopathy.

Adolescent↗

A clinical role for right ventricular endomyocardial biopsy.

There is considerable uncertainty about the value of endomyocardial biopsy (EMB) in the diagnosis and management of patients with suspected primary myocardial dysfunction. To determine the clinical utility of this procedure in patients referred to our centre, we reviewed the clinical records and biopsy findings of the first 21 consecutive patients in whom we performed right ventricular EMB. Patients were divided into four groups according to the clinical indications for EMB: unexplained congestive cardiac failure and a dilated heart (Group 1: 11 patients); unexplained congestive cardiac failure and a non dilated heart (Group 2: three patients); unexplained cardiomegaly in the absence of cardiac failure (Group 3: one patient); suspected hypertrophic cardiomyopathy (HCM) (Group 4: six patients). Histological examination of EMB tissue obtained from all patients in Group 1 as well as the single patient in Group 3 showed non specific features judged to be compatible with a diagnosis of dilated cardiomyopathy. Accordingly, in all patients in Groups 1 and 3, a potentially treatable cause of primary myocardial dysfunction was excluded. Biopsy examination demonstrated the presence of a specific disease process in two of three patients in Group 2 (one patient had amyloidosis, the other endomyocardial fibrosis). In five of the six patients in Group 4, the biopsy findings were either diagnostic or suggestive of HCM. Our results suggest that EMB is a clinically useful tool in patients presenting with features suggestive of a primary myocardial disorder.

Biopsy↗

Effective therapy for a severe case of the idiopathic hypereosinophilic syndrome.

An 8-year-old boy with idiopathic hypereosinophilic syndrome (HES) is reported. He has been maintained in prolonged hematologic remission with vincristine and mercaptopurine despite an initial eosinophil count of 186 X 10(9)/L and a poor response to prednisone and hydroxyurea. Success with this combination of chemotherapeutic agents has not previously been reported in the management of HES. Progressive endomyocardial fibrosis required cardiac surgery 11 months after diagnosis. The management of this disorder is discussed with a review of the published literature.

Cardiomyopathies↗

The status of cardiovascular surgery in West Africa.

This report reviews the present status of cardiovascular surgery in West Africa and highlights some of the constraints of development in this field.Rheumatic heart disease is still endemic in the tropics, where it constitutes about 20 percent of all cases of cardiovascular disease (CVD) in Nigeria. Endomyocardial fibrosis is a disease of unknown etiology accounting for 10 to 20 percent of cases. Purulent pericarditis is a common complication of pyomyositis and osteomyelitis found in 5 percent of patients. Chronic constrictive pericarditis is a sequela of infective pericarditis found in 5 percent of all cases of CVD. Calcification is found in 30 percent of cases and pericardiectomy can be performed successfully without cardiopulmonary bypass. Infective endocarditis is equally rare, occurring in 2.5 percent of cases; it is a common source of septic emboli to coronary artery and a very difficult disease to treat in the West African environment.Ischemic heart disease is relatively uncommon, accounting for less than 0.5 percent of patients. The rarity of the disease in black Africans has been attributed to dietary habits and environment rather than to racial and psychosocial factors. Congenital heart disease accounts for 5 percent of all cases of CVD in this review. Ventricular septal defect and patent ductus arteriosus are the most common acyanotic defects, while tetralogy of Fallot and transposition of the great arteries are the most common cyanotic defects.Vascular diseases are uncommon in this series, with traumatic injuries accounting for most of the cases. Abdominal aortic aneurysms, peripheral occlusive vascular disease, and atherosclerotic aortic aneurysms are quite rare. This review further confirms the rarity of deep venous thrombosis and pulmonary embolism in Africans.

Adolescent↗

Etiology of pure tricuspid regurgitation.

Of multiple etiologies for pure tricuspid regurgitation, the causes may be divided into those associated with anatomically abnormal and anatomically normal tricuspid valves. Conditions associated with anatomically abnormal valves include rheumatic, floppy, Ebstein's anomaly, carcinoid, infective endocarditis, papillary muscle dysfunction, and other rarer causes such as radiation, hypereosinophilic syndrome, and endomyocardial fibrosis. Conditions associated with anatomically normal tricuspid valves include multiple causes of cor pulmonale (chronic obstructive pulmonary disease, primary pulmonary hypertension, and pulmonary hypertension due to mitral stenosis). Of 45 necropsy patients with clinically documented pure tricuspid regurgitation, conditions producing pulmonary hypertension (cor pulmonale, mitral stenosis) were the most frequent etiology (47 percent), followed by floppy (16 percent), rheumatic (11 percent), idiopathic dilated cardiomyopathy (9 percent), Ebstein's anomaly (7 percent), and miscellaneous conditions (10 percent). Of multiple causes of pure mitral regurgitation, tricuspid valve anular circumference and leaflet area are useful measurements in establishing etiology: dilated anuli (greater than 12 cm) are associated with Ebstein's anomaly, floppy valves, and multiple causes of pulmonary hypertension. Increased leaflet areas are associated with floppy valves and Ebstein's anomaly. Anular insertion site separates floppy tricuspid valves from Ebstein's anomaly. Of 21 purely regurgitant operatively-excised tricuspid valves, the etiologies were: rheumatic (43 percent), "functional" due to pulmonary hypertension from mitral stenosis (38 percent), Ebstein's anomaly (9 percent), infective endocarditis (5 percent), and floppy (5 percent). In contrast to etiology of pure mitral regurgitation, rheumatic disease is the leading cause for operative excision of purely regurgitant tricuspid valves. Comparison of systolic pulmonary arterial pressures and tricuspid valve morphology disclosed that pulmonary arterial pressures greater than or equal to 55 mm Hg and tricuspid valve anuli greater than 12 cm were associated with anatomically normal tricuspid valves and that pulmonary arterial pressures less than or equal to 40 mm Hg and tricuspid anuli less than 12 cm were associated with anatomically abnormal tricuspid valves.

Adolescent↗

[Open-heart valvular surgery in children. Apropos of 127 cases].

In view of the frequency and the severity of rheumatic heart disease (RHD) and of endomyocardial fibrosis (EMF) in the african child, the indication of a valvular surgical procedure is frequent in Abidjan. Between 1978 and 1983, 127 open-heart procedures have been performed in 113 patients. The ages ranged from 2 to 15 years, with a mean of 11 years. There were 67 RHD, 37 EMF, 9 bacterial endocarditis. The status of the patients was severe, there was a cardiomegaly (mean CTR of 0.70). 3 patients were in atrial fibrillation. The patients underwent 97 valve replacements (70 mitral, 18 tricuspid, 9 aortic) essentially by a bioprosthesis (n : 87). A mitral valvuloplasty was done in 27 cases. Associate procedures have been employed including : 35 endocardectomies, 11 aortic valvuloplasties, 6 tricuspid annuloplasties. 12 patients have been reoperated for bacterial endocarditis of a mitral prosthesis (n : 4), calcification of a mitral bioprosthesis (n : 5), failure of mitral valvuloplasty (n : 3). There were 13 deaths (10.2 p. 100 mostly in EMF surgery : 7 deaths (5.5 p. 100). No patient with a bioprosthesis received anticoagulants post-operatively. All surviving patients have been improved, with a mean post-operative follow-up of 32 months. There were 11 late deaths (8.6 p. 100). The authors discuss several points including : severity of children valvular diseases in tropical areas; necessity to use the bioprosthesis in this area of the world; increasing tendency to conservative surgery in the mitral and also the aortic position.

Adolescent↗

Clinical, hemodynamic and endomyocardial biopsy findings in idiopathic restrictive cardiomyopathy.

Restrictive cardiomyopathy is uncommon and in its overt form is associated with heart failure, characterized primarily by abnormalities in diastolic function and preserved or nearly preserved systolic function. It may be associated with amyloidosis, hemochromatosis or endomyocardial fibrosis. We describe five patients with restrictive cardiomyopathy, ages ranging from 35 to 71 (mean 49), three of whom were men. Fatigue, dyspnea on exertion and chest pain were the most frequent symptoms. Only one patient had overt heart failure, and three had normal or near-normal hemodynamics at rest that became greatly abnormal with exercise. Four of the five patients are alive now 9 to 77 (mean 33) months following the onset of symptoms. Despite prior emphasis on specific causes, restrictive cardiomyopathy in this series had no definable cause. Moreover, the presence of a "latent" form of restriction (abnormalities only with exercise) suggests that the incidence of the disease may be higher than previously appreciated.

Adult↗

Multifocal myocardial necrosis and fibrosis in pancreatic diseases of children.

From a review of 2,000 autopsies of children, 16 cases of extensive necrosis and scarring fibrosis of the myocardium were found. These lesions involved mainly the left ventricle and spared the endocardium, the pericardium, and the coronary vessels. These necrotic of fibrotic heart lesions were found to be closely associated with various pancreatic diseases: cystic fibrosis (11 cases), pancreatic lipomatosis (2 cases), extensive small bowel resection (3 cases, 2 of which were associated with acute interstitial pancreatitis). To explain these unexpected associations, two hypotheses can be put forth: (1) The lack of absorption of some presently undetermined substances indispensable for the correct trophicity of the myocardium, and (2) the release in the blood of proteolytic enzymes with consecutive activation of phlogistic substances such as kinins.

Acute Disease↗