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[Two cases of endomyocardial fibrosis in Mozambique].

This essay describes two cases of Endomiocardial Fibrosis with right ventricular dominance studied in Mozambique. The clinical, radiological, echocardiographic and patological findings are in accordance with other descriptions of same disease, seen in others countries.

Adolescent↗

The role of thorium in endomyocardial fibrosis.

The behaviour of thorium in animals exposed through injection, ingestion and inhalation, as well as in uranium miners, millers and the general population suggests that thorium accumulates primarily in the lung, tracheobronchial lymph nodes and skeleton; or in the liver if injected with polymeric thorium. Epidemiological studies on uranium miners, Thorotrast studies in man and animal, and studies on the toxic effects of thorium in animals suggest that there is no apparent heart diseases due to exposure to thorium in man and animal.

Animals↗

Studies on the experimental production of endomyocardial fibrosis and cardiomegaly of unknown origin by dietary means.

Three groups of rats were kept on diets similar to those eaten by indigenous inhabitants in parts of Africa where CUO and EMF are commonly found, for 30 months. Many of the animals on a tryptophan-deficient diet developed CUO whereas those on a tryptophan-deficient diet which contained a 5-HT content also developed lesions similar to EMF. The third group of rats, which ate a diet similar to this second group but supplemented by a high-protein intake, did not develop any cardiac lesions.

5,6-Dihydroxytryptamine↗

Maximal functional capacity in patients with endomyocardial fibrosis.

We evaluated objectively the cardiocirculatory dysfunction of patients with EMF, and related the functional class (FC-NYHA) to the data obtained by analysis of maximal functional capacity. We studied 55 subjects, divided into three groups: group 1 (G1) 21 (38.1%) normal persons, group 2 (G2) seven (12.7%) patients with EMF who were in FC I or II, and group 3 (G3) 27 (49%) patients in FC III or IV. Maximal oxygen consumption (VO2max) and O2 pulse index (PO2max) were obtained using a Beckman computerized gas analyser. The mean values were significantly different among the three groups. We conclude that the greater the clinical impairment, the greater the alterations that occur in the indices of cardiac function.

Adult↗

Role of macrophages in the pathogenesis of endomyocardial fibrosis in murine malaria.

C57B1/Rij mice developed progressive endocardial oedema and extensive endocardial thrombosis with a predilection for the right half of the heart in the course of a lethal Plasmodium berghei infection. Chemotherapy of an ongoing infection resulted in fibrosis of affected areas. Despite a close correlation between development of lesions and parasitaemia, parasitized erythrocytes were not usually present in the affected areas. Macrophages might play, however, an important role in the pathogenesis of the lesions. Early changes included sticking of macrophages to the endocardial endothelium, migration to subendothelial areas associated with leakage, and oedema. Subsequently, subendothelial infiltrates of lymphocytes, neutrophilic granulocytes, macrophages and fibroblasts were found. Moreover, endothelial lesions, sometimes associated with cell migration, were found to be plugged by microthrombi. Mural thrombi grew out eventually until death of the host or until chemotherapy cleared the infection.

Animals↗

Endomyocardial fibrosis masquerading as rheumatic mitral incompetence. A report of six surgical cases.

This report presents six cases (among 43 surgical cases) of left ventricular EMF presenting as pure mitral insufficiency without any echocardiographic or hemodynamic signs of left ventricular EMF (except apical diverticulum in two cases). Five of these cases were diagnosed at operation, and the sixth was diagnosed on the basis of a characteristic right ventricular angiogram. At operation a fibrotic lesion localized to the level of the anteropapillary muscle, with severe mitral insufficiency, was found. Five patients underwent successful mitral valve replacement and one a successful conservative mitral valve procedure. Postoperative angiograms, done in two patients, showed a normal contour of the left ventricle. A review of the literature did not reveal any previous descriptions of this type of limited left ventricular EMF, mimicking rheumatic mitral insufficiency. In our surgical experience, we have encountered three types of left ventricular EMF: obliterative, extensive, and limited. We emphasize EMF as a possible cause of mitral insufficiency in African children.

Adolescent↗

Degranulated eosinophils, eosinophil granule basic proteins and humoral factors in Nigerians with endomyocardial fibrosis.

The frequency of eosinophilia, degranulated eosinophils, and raised serum levels of eosinophil granule basic proteins was determined in ten Nigerians with EMF and fifteen normal controls matched for age and sex, and from the same environment and social background as the patients. Sera from the patients and control subjects were also examined for auto-antibodies, immune complexes, filaria antibodies and immunoglobulins. A mild eosinophilia was observed in four of the patients. The mean direct eosinophil count in the patients (0.407 +/- 0.14 X 10(9)/l) was, however, not significantly different from that of the control subjects (0.399 +/- 0.07 x 10(9)/l). Degranulated eosinophils were absent in the blood and bone marrow aspirates of the patients, and peripheral blood of the controls. It was also found that the mean concentration of eosinophil granule basic proteins (ECP/EPX) in the patients (0.278 +/- 0.1 micrograms/ml) was not significantly different from the mean value for the control subjects (0.371 +/- 0.1 micrograms/ml, P greater than 0.5). Serological studies using Brugia pahangi antigens failed to demonstrate filaria antibodies in the patients and the control subjects. Microfilaria were also absent in their peripheral blood films. Using the indirect immunofluorescent technique, anti-heart antibodies were not demonstrated in the sera of the EMF patients and control subjects. Five (50%) of the patients and four (30.7%) of the normal controls had immune complexes levels far in excess of the upper limit of normal. However, the mean concentration of immune complexes in the patients (16.35%) was not significantly different from the value for control subjects (12.13%, P greater than 0.5).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Endomyocardial fibrosis: surgical treatment].

The authors analyse the experience acquired for surgical treatment of endomyocardiofibrosis. Among the 15 patients submitted to the operation, nine had the biventricular form, four had the disease in the right ventricle and two only in the left ventricle. The surgery consisted of endocardioectomy of the ventricles (RV--04,LV--02, bilateral--08) and replacement of the diseased atrioventricular valves (mitral--02, tricuspid--06, mitral and tricuspid--07). In one patient there was severe obstruction of the anterior descending artery and a bypass was done. Three patients had associated rheumatic mitral stenosis. There was one early operative death and three late deaths (respectively 13.34 and 37 months later). Two patients required re-operation. The first one, 33 months later, to replace the mitral valve which had been preserved in the first procedure, and the second, 12 months later, who presented ventricular septal defect secondary to rupture of the weak septum. Of all the patients who underwent surgery, 11 survived and all, except one, have had a good course. The detailed analysis of this series permitted us to conclude that: 1st--the general use of the bidimensional echocardiography in high risk patients, allows early diagnosis in a greater number of them; 2nd, the surgery must be early to avoid cardiac and extracardiac injury due to the disease; 3rd--a low profile valve must be used; 4th--to this moment there have been no signs of recurrence; 5th--surgical treatment appears to improve the course of this terrible disease.

Adolescent↗

Early surgical results in the treatment of endomyocardial fibrosis. A Brazilian cooperative study.

This report summarizes the surgical experience and early results obtained at four surgical centers in the northeast and south of Brazil. From December 1977 to September 1986, 95 operations were performed on 93 patients, ages 11-59. Bilateral lesions occurred in 42 patients, right lesions in 39, and left lesions in 12. Ventricular decortication and removal of thrombi were performed in all. In right-sided lesions, the tricuspid valve was substituted by a bioprosthesis in 34 cases, and substituted by a tilting disk valve in 1 case. In 4 patients, the valve could be preserved. The left-sided lesions led to valve substition by a bioprosthesis in 11 cases, and preservation of the valve in 1. The bilateral lesions needed bioprosthesis in the mitral position in 37 patients, and a disk valve in 2. In these 39 instances, the valvular procedure was insertion of a tricuspid bioprosthesis. Three tricuspid and three mitral plasties were performed. The overall mortality was 20% (26.2% for bilateral lesions, 14.6% for the right-sided lesions, and 20% for the left-sided lesions). The main cause of death was low cardiac output. Aside from a variable degree of right and left ventricular failure, many other non-fatal complications clouded the postoperative course. Complete AV blocks occurred in 10 cases, with the need for permanent pacing in 7 survivors. The mortality and morbidity in the present series is in keeping with the results reported in current literature. Regarding the advanced stage of their patients' disease, the authors agree with the recommendation for earlier surgical intervention.

Adolescent↗

Simulation of acute myopericarditis by constrictive pericardial disease with endomyocardial fibrosis due to methysergide therapy.

Methysergide ( Sansert ) has been associated with numerous fibrotic disorders. In particular, multiple cardiac lesions have been described in cases where methysergide was thought to have played a causative role. A patient is described who presented with cardiac findings suggestive of acute myopericarditis . An inflammatory myocarditis was subsequently excluded by endomyocardial biopsy. Hemodynamic findings suggested the presence of constrictive pericarditis or restrictive cardiomyopathy, or both. Radiographic evidence of constrictive pericarditis and biopsy evidence of endocardial fibrosis were documented in this patient with a long history of interrupted methysergide therapy.

Acute Disease↗