Gross pericardial effusion in endomyocardial fibrosis.
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Cardiac involvement frequently occurs in hypereosinophilic syndrome. The endocardium seems to be most susceptible to disturbances deriving from eosinophilia as seen in Loeffler's endocarditis. Hypereosinophilia and cardiac involvement are also seen in Churg-Strauss syndrome. The present report deals with a patient with Churg-Strauss syndrome who exhibited marked endomyocardial fibrosis that was visualized by cardiac magnetic resonance imaging. Loeffler's endocarditis is the classical endocardial manifestation of hypereosinophilic syndrome. Similarities between Loeffler and Churg-Strauss syndromes had been described previously. Potentially, endocardial involvement is more common even in Churg-Strauss syndrome than expected so far. The significance of these findings remains to be shown.
Chronic thromboembolic pulmonary hypertension is a rare sequela to an acute untreated or recurrent pulmonary embolism. The mechanisms that underlie the failure to resolve the thrombus are still uncertain. As most patients are not diagnosed until a relatively late stage, little is known about the course of their illness. We report the case of a 51-year-old woman who had previously been diagnosed with and operated on for endomyocardial fibrosis of the right ventricle and who developed chronic thromboembolic pulmonary hypertension several years later.
A case of 42-year-old female with persistent pericardial effusion and recurrent congestive heart failure was presented. The clinical course, laboratory and cardiac evaluations confirmed the existence of tricuspid incompetence and restrictive condition of the right ventricle. At autopsy, the right atrium and ventricle showed moderate hypertrophy, the left ventricle being almost completely spared macroscopically. Diffuse fibrous thickening of the right atrial and ventricular endomyocardium with mural thrombi, and mild lymphocytic infiltation were noted microscopically. These findings are compatible with endomyocardial fibrosis described by Davies. The etiology and pathogenesis of the disease were discussed.
The surgical technique of endocardiectomy and valve replacement for patients with uni- or biventricular endomyocardial fibrosis is described. Technical details of the operation are outlined, based upon experience with 37 surgical cases.
A man of 54 had been treated over a 15 month period for pulmonary tuberculosis when aged 41, and had lived on the Ivory Coast and then in the Cameroons for 20 years. Eye signs were discovered in January 1971, and these, together with an eosinophilia, pointed to a possible filariasis which was later confirmed by immunofluorescence and the fixation of complement. Three months later, the patient developed congestive cardiac failure. On the 4th day of treatment with Notizine, multiple systemic emboli occurred, leading to death. Autopsy showed endomyocardial fibrosis with multiple ulceration of the left ventricle, the site of the emboli. The aetiology from filariasis and the mechanism of ulceration of the parietal endocarditis are discussed.
The patient was a 29-year-old woman. When she consulted a local physician with chief complaints of fever and fatigue of the extremities, cerebral infarction was detected on MRI, in addition to abnormalities found on ECG. Ultrasonic cardiography revealed the presence of a tumor in the left ventricle. Therefore, tumorectomy and endocardectomy were performed under extracorporeal circulation based on a diagnosis of cardiac tumor. Inflammatory cell infiltration into the ventricular wall was pathologically confirmed, and eosinophilia was observed preoperatively. Therefore, the patient was diagnosed as having endomyocardial fibrosis, which is rarely observed in Japan. The postoperative course of this patient was satisfactory, and the eosinophil count was normalized postoperatively. At present, this patient is being followed at the outpatient clinic.
A 66-year-old man with an ECG finding of an elevation of R in leads V5-6 and a calcified space occupying lesion in the apex of the left ventricle on echocardiography admitted for the evaluation of recent onset of shortness of breath and palpitation on exertion. Cineangiography showed an obliteration and accumulation of RI activity in the apex of the left ventricle on TI-201 scintigraphy and a neoplasm of the heart was suspected. Resection of a tumor from apical incision followed by the left atrial approach necessitated the replacement of mitral valve and confirmed the diagnosis of endomyocardial fibrosis. Heart failure persisted and the expired 2 years after surgery due to the gastrointestinal infection. An experience suggested the resection of a mass from the left atrial approach is possible and recommended to prevent the occurrence and persistence of heart failure in case the diagnosis is established preoperatively.
A 32-year-old man presented with left hypochondrial pain and myalgia. On examination he was found to have widespread lymphadenopathy and splenomegaly, and lymph node biopsy revealed diffuse lymphocytic lymphoma of the small cleaved cell type. There was hypereosinophilia (20 x 10(9)/1). The patient was initially treated with chlorambucil and prednisone, which controlled the lymphoma for 9 months but did not affect the eosinophilia. He then developed thrombocytopenia with further lymph node enlargement which was managed with a combination of bleomycin, vincristine and prednisone; this again controlled both spleen and lymph node size without affecting the eosinophilia. Six months after presentation the patient developed refractory cardiorespiratory failure due to endomyocardial fibrosis and fibrosing alveolitis. The cardiac failure was thought to be related to the hypereosinophilia resulting from the lymphoma, while the respiratory insufficiency was attributed to the bleomycin. This case illustrates the rare association between lymphocytic lymphoma and eosinophilia and shows that cardiac damage can be associated with these cells.
On epidemiological basis EMF behaves like a vector transmitted disease. The cardiac pathologies of EMF and HES are identical. In some cases of HES, hypereosinophilia may return to normal, leaving residual heart disease that is exactly like EMF. Most temporary residents from Europe and North America who developed EMF while resident in the endemic areas of Africa had hypereosinophilia that was induced by helminths. In our case studies from the EMF endemic areas of Nigeria, most children with acute idiopathic myocarditis associated with helminth induced hypereosinophilia, developed clinical EMF on follow up. We showed also that the rate of decline in the incidence of hypereosinophilia in EMF cases was significantly related to the duration of symptoms. Our studies and other observations show that EMF, like HES is a multiple system disease with similar organ damage. The morphologic evolution of cardiac damage in EMF appears similar to that reported for HES; with a stage of myocarditis/pericarditis, followed by a stage of cardiac necrosis, a stage of thrombosis and by the chronic fibrotic stage. Also during larval migration, all the helminths associated with EMF induce the same spectrum of damage in the central and peripheral nervous system, in the lungs, kidneys and skin, as are reported for HES. The cardiovascular damage reported for these worms (which include hypersensitivity vasculitis, acute myocarditis/ pericarditis) are also similar to what is reported for HES. Acute endomyocardial necrosis and thrombosis that are similar to what is found in HES, have been documented in Trichinella Spiralis and in filariasis. Increased cerium concentrations have been documented in the endocardium of EMF cases from South India. It remains to be established whether cerium excess, which is known to stimulate collagen synthesis does accelerate the process of endomyocardial fibrosis, following cardiac necrosis (which may have been triggered by helminths and the associated hypereosinophilia).
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Explore the source record for details and available documents.
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Explore the source record for details and available documents.
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Restrictive cardiomyopathies have been variously classified and interpreted. Although their etiologic and anatomic features may vary, their common denominator is indicated by restrictive hemodynamic behavior of either the left or right ventricle or both. This report describes a case in which such restriction was caused by obliterative endomyocardial fibrosis of unknown etiology. Because of significant symptomatology, including congestive failure and syncope, the patient underwent surgical excision of the endomyocardial overgrowth.