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Results for “ENDOMYOCARDIAL FIBROSIS”

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PubMed1983

Endomyocardial fibrosis in Africa.

Endomyocardial fibrosis (EMF) is a disease of the rain-forest belt in Africa. There is general agreement as to its pathology in the acute phase, but this is difficult to diagnose clinically. The aetiology is still unknown although there are reports which suggest that eosinophilic endomyocardial disease may be the cause. Further studies are needed to define EMF in its acute stage and find out how chronic EMF evolves. A longitudinal study on young people with eosinophilia and a comparative study of two villages, one in an endemic zone and the other in a zone where EMF is uncommon, will also be helpful in identifying its cause. The most promising form of treatment at present is surgical.

Africa↗

PubMedDate not supplied

[Doppler echocardiography in endomyocardial fibrosis].

Twelve patients with endomyocardial fibrosis with angiographic and/or histologic corroboration were studied with Doppler echocardiography with the purpose of describing the echocardiographic features and identify the affected sites. The average age was 41 years (range 16 to 59 years), 2 men and 10 women. Three patients (25%) had isolated right ventricular involvement, one patient (8%) left ventricular, 8 patients (66%) both ventricular. Our Doppler echocardiographic findings were: right atrium enlargement (91%), right ventricle outflow dilatation (83%), paradoxical septal motion (83%), left atrial enlargement (33%), mitral and tricuspid valve prolapse (50%), pericardial effusion (41%), mitral regurgitation (75%), tricuspid regurgitation (100%), apex obliteration (50%) and a restrictive type flow pattern (50%). Doppler echocardiography is a useful method for the diagnosis of endomyocardial fibrosis, the finding of normal or small ventricles associated with apex obliteration and enlarged atria, mitral or tricuspid regurgitation and a restrictive type flow pattern are characteristics of this disease. In our population, the isolated or predominantely right ventricular involvement is the most common finding as it represented 83% of the cases.

Adolescent↗

PubMed1986

A geochemical basis for endomyocardial fibrosis.

In a search for geochemical factors that could play a role in the pathogenesis of tropical endomyocardial fibrosis, endomyocardial tissue samples obtained from patients at necropsy or operation were analysed for major elements present in laterite and monazite, which are important soil constituents of Kerala State of India. Atomic absorption spectrophotometry was used for detecting iron, silicon, aluminium, zinc, magnesium, calcium, sodium, potassium, and manganese and neutron activation analysis for thorium. Compared with control samples from victims of fatal accidents, an excess of thorium, sodium, and calcium and a deficiency of magnesium were present in samples from patients. It has been shown earlier that the staple diets of people in Kerala have high concentrations of thorium, and these data show that thorium can become concentrated in cardiac tissues. It is speculated that thorium excess in conjunction with magnesium deficiency may play a role in the causation of tropical endomyocardial fibrosis.

Endomyocardial Fibrosis↗

PubMed1985

Endomyocardial fibrosis: report of eight cases.

Endomyocardial fibrosis is a disease of unknown origin which has not previously been described in detail from the Middle East. The clinical, echocardiographic, hemodynamic and angiocardiographic findings in eight patients (five men and three women, mean age 38 years) are presented. Two patients had right-sided involvement, two had left-sided involvement and four had biventricular involvement. The presence of a small ventricle with obliteration of the apex and a large atrium is a two-dimensional echocardiographic finding highly suggestive of endomyocardial fibrosis. Hemodynamic characteristics of dip and plateau on ventricular pressure curves were present in six patients. Ventricular angiography was diagnostic in all cases. Endomyocardial biopsy yielded positive findings in three of six patients and is not essential for diagnosis.

Adult↗

PubMedDate not supplied

Echocardiographic recognition of Chagas' disease and endomyocardial fibrosis.

Chagas' heart disease and endomyocardial fibrosis are common medical conditions in Central and South America but are only rarely encountered in North America. In the small number of patients who have these conditions, recognition is frustrating because of a lack of familiarity with their characteristic echocardiographic pattern. In Chagas' heart disease a left ventricular apical aneurysm is characteristic, but in contrast to coronary artery disease, septal involvement is minimal. In endomyocardial fibrosis apical obliteration with a small inwardly moving left ventricular cavity, large atria, and atrioventricular valvular insufficiency are typical features. It is the aim of this article to present the characteristic echocardiographic findings with these conditions and thereby facilitate the recognition when they appear in nonendemic areas.

Adult↗

PubMed1977

Endomyocardial fibrosis and eosinophilia.

Absolute eosinophil counts were assessed in 15 African patients with proven endomyocardial fibrosis. Though the mean eosinophil count in patients with endomyocardial fibrosis was higher compared with the normals reported from Kampala (1-13 vs 0.72X10(9)/1), the absolute range was comparable. A high percentage of patients with endomyocardial fibrosis had malarial parasites, high malarial antibody titres, hookworms, or strongyloides, but the correlation of eosinophilia to various parasitic infections was poor. Both eosinophilia and parasitic infections are common in the tropics and they effect patients with endomyocardial fibrosis no more than the population at large. Other aetiological factors, genetic, environmental, and immunological, are felt to be important in the causation of endomyocardial fibrosis in Uganda and evidence for this is reviewed. Though there is a similarity in pathological features, African endomyocardial fibrosis is a distinct entity from Löffler's endocarditis and cardiac lesions seen in eosinophilic leukaemia or reactive eosinophilia. There is no hard evidence to suggest that African endomyocardial fibrosis is a variant of Löffler's endocarditis caused by parasitic infections via eosinophilia.

Adolescent↗

PubMed1988

Unusual presentation of endomyocardial fibrosis.

A case of endomyocardial fibrosis in a Greek patient who had not visited the tropics is described. The patient suffered from an unusual form of the disease in that it was aggressive and recurrent (requiring three operative procedures) and presented with tricuspid stenosis, leading to syncope.

Adult↗

PubMed1989

Endomyocardial fibrosis in the child.

Endomyocardial fibrosis is a rare disease in children and has been mainly observed in tropical Africa, seldom in Europe. Its precise aetiology remains unknown. New surgical procedures have recently led to better survival. Four children, a 5 year old Swiss boy and 3 African girls, aged 12 to 14 years, were submitted with global heart failure class III to IV of the NYHA classification. Two patients had eosinophilia on admission. A third had a history of transient eosinophilia. The echocardiographic examination showed a very typical picture. In 3 patients (2 f and 1 m) both ventricles were involved; in one girl only the left ventricle. Decreased ventricular distensibility with impaired filling of the left and/or right ventricle was present in all. Diastolic pressures ranged between 24 and 35 mmHg; the systolic function was satisfactory in 3 children (ejection fraction [EF] of 44 to 61%) and severely decreased in the fourth (EF 10%). Three patients underwent endocardectomy: both ventricles in 1 case, only the left in 2 cases. Mitral valve repair by means of the Carpentier ring was done once, valve replacement once (Starr-Edwards prothesis). One child died; the 2 surviving patients showed a distinct improvement in their clinical state and are now in class II of the NYHA classification. Echocardiographic and catheter investigations show, however, persistence of the restrictive cardiomyopathy. Thus, surgical intervention must be considered a palliative procedure in these cases. The diagnosis can clearly be made nowadays with bidimensional and Doppler echocardiography.

Adolescent↗

PubMed1977

Löffler's endocarditis and endomyocardial fibrosis--a nosologic entity?

The problem whether Löffler's endocarditis and endomyocardial fibrosis belong to the same disease spectrum or are separate entities is still under debate. Until recently it was believed that endomyocardial fibrosis was a disorder restricted to tropical areas. Three Danish patients are presented, two showing a continuous disease spectrum, one patient showing the fully developed endomyocardial fibrosis which is indistinguishable from endomyocardial fibrosis described from the tropical areas. Eosinophilia was present in all three patients. The findings described in these three patients lend support to the unitarian hypothesis that Löffler's endocarditis and endomyocardial fibrosis belong to the same disease spectrum, and that the eosinophilic granulocyte may be the underlying cause.

Adult↗

PubMed2001

[Tropical endomyocardial fibrosis or Davies disease. A case report].

Endomyocardial fibrosis is a restrictive endomyocardial myocardiopathy of unclear etiology, it is an endemic disease in tropical and subtropical countries. The patients are usually young and the disease has an insidious onset and poor prognosis. Surgery may lead to clinical improvement and prolong life expectancy, although it is only a palliative treatment and does not stop disease progression. We present a case of endomyocardial fibrosis from Equatorial Guinea with severe clinical involvement and clear improvement following surgery. The current etiopathogenic hypothesis, the results obtained and the management of these cases, which are infrequent in Spain, are discussed. We also comment upon the differences with the historically related Loeffler syndrome according to one of the etiopathogenic hypotheses.

Adult↗

PubMed1997

Endomyocardial fibrosis in Behçet's disease.

OBJECTIVE: To report on four patients with Behçet's disease associated with endomyocardial fibrosis involving the right or the left ventricle. METHODS: Charts of more than 350 patients with Behçet's disease were reviewed. Endomyocardial fibrosis was confirmed because of cardiac failure in three patients and incidentally discovered by histological examination of an operative specimen in one patient. Echocardiography displayed bright echogen endocardium. Angiocardiography showed a reduced ventricular size. Electron beam computed tomography demonstrated a lowdense area involving the endocardium. Magnetic resonance imaging showed a mass of intermediate intensity on T1 weighted images. Diagnosis of endomyocardial fibrosis was based on histological study of a biopsy specimen in one patient and of an operative specimen in three. RESULTS: Six other similar cases of endomyocardial fibrosis complicating Behçet's disease were previously reported in the medical literature. Endomyocardial fibrosis predominantly involved the right ventricle. It can be considered a feature of Behçet's disease because: (a) no other cause was discovered; (b) arteritis, valvulopathy, and intraventricular thrombus were closely linked, and (c) all patients with endomyocardial fibrosis had vasculo-Behçet pattern. CONCLUSION: Endomyocardial fibrosis may be the sequelae of vasculitis involving endocardium or myocardium, or both and complicated with intraventricular thrombosis. Behçet's disease should be added to the list of causes of endomyocardial fibrosis.

Adult↗

PubMed1986

Postoperative evaluation of endomyocardial fibrosis.

25 patients with angiographic features suggestive of endomyocardial fibrosis underwent endocardiectomy and atrioventricular valve replacement. There were 8 deaths. 17 survivors were reevaluated after a mean follow-up of 13.5 months. 8 had right ventricular endomyocardial fibrosis (RVEMF) 5 had left ventricular endomyocardial fibrosis (LVEMF) and 4 had biventricular disease (BVEMF). The survivors showed a significant improvement in their clinical features and functional status. There was a significant reduction in atrial size in X-ray. Hemodynamic data showed a significant fall in right heart filling pressure, right ventricular systolic pressure and pulmonary artery mean pressure, and a significant fall in left heart filling pressure in patients with left ventricular disease either alone or as a part of biventricular disease. In ventriculogram, while there was no change in right ventricular shape after endocardiectomy, left ventricular shape was restored to near normal. The significant improvement is mostly due to the correction of the regurgitant lesion and to some extent due to restoration of ventricular compliance.

Adolescent↗

PubMed1982

M-mode echocardiographic features of endomyocardial fibrosis.

M-mode echocardiographic findings are described in 21 patients with endomyocardial fibrosis. Features associated with right ventricular endomyocardial fibrosis include: (i) exaggerated motion and thickening of the anterior right ventricular wall; (ii) increased right ventricular end-diastolic dimension; and (iii) paradoxical septal motion. Pericardial effusion (viz an echo-free space behind the posterior left ventricular wall) was shown in three patients. The tricuspid valve was easily recorded in all. Six patients with left ventricular endomyocardial fibrosis had diminished left ventricular end-diastolic dimension. Three had echo features of pulmonary hypertension (viz reduced e-f slope, absent a wave in sinus rhythm, and systolic notching of the pulmonary valve echogram). Fine fluttering of the anterior mitral valve and tricuspid valve echo was observed in two patients (one of whom was in sinus rhythm) with biventricular endomyocardial fibrosis, and no angiographic evidence of aortic regurgitation.

Adolescent↗

PubMedDate not supplied

Endomyocardial fibrosis is associated with selective deposition of type I collagen.

BACKGROUND: Endomyocardial fibrosis is a distinct form of heart disease leading to restrictive ventricular filling and cardiac failure. The disease is characterized by a marked thickening of the endocardium due to the deposition of dense fibrous tissue composed of wavy bundles of collagen. Changes in collagen composition and an abnormal increase in its concentration result in a stiffer myocardium and ventricular diastolic dysfunction. The nature of cardiac collagens and the relative proportions of collagen types in endomyocardial fibrosis have not been documented in the literature. METHODS AND RESULTS: This study analyzed collagen composition in the cardiac tissues of 13 patients with endomyocardial fibrosis and 6 individuals who were the victims of traffic accidents or suicidal deaths and did not have any heart disease. We estimated the relative proportions of types I and III collagen after pepsin digestion of the tissue and separation of the emerging peptides by sodium dodecyl sulfate polyacrylamide gel electrophoresis. The mean type I:III collagen ratio was 0.51+/-0.06 in normal individuals, and 0.93+/-0.43 in patients with endomyocardial fibrosis (p<0.05). The alteration in the type I:III collagen ratio was due to a disproportionate increase in type I collagen. CONCLUSIONS: The results indicate that a selective increase in type I collagen may contribute to the impaired diastolic distension of the ventricles in patients with endomyocardial fibrosis.

Adult↗

PubMed2004

Assessment of diastolic function in endomyocardial fibrosis: value of flow propagation velocity.

Endomyocardial fibrosis is manifested mainly by diastolic heart failure. However, diastolic function has not been well characterized in this disease. The aim of this study was to characterize left ventricular (LV) diastolic function in endomyocardial fibrosis (EMF) by echocardiography. Eighteen patients with LV EMF and 18 healthy subjects were studied. Cardiac volumes and ejection fraction were assessed by Simpson's method. Pulsed-wave Doppler was used to obtain mitral and pulmonary venous flow velocities and grade diastolic function. Pulsed-wave tissue Doppler imaging velocities along the septal side of mitral annulus, flow propagation velocity (Vp) of the early diastolic mitral inflow, and myocardial performance index were obtained. According to this grading method, four patients with normal diastolic function, five with impaired relaxation, five with pseudonormal, and four with a restrictive pattern were found. A positive correlation of these diastolic function grades and NYHA functional class was found (r = 0.66, P = 0.003). By "stepwise" logistic regression the best index that discriminated EMF patients from controls was Vp. The probability of occurrence of EMF = exp(7.9288 - 0.1366 Vp)/1 + exp(7.9288 - 0.1366 Vp). A wide range of diastolic function grades is found in patients with EMF and these correlated with functional class. Delayed myocardium relaxation, as reflected by altered Vp, was a frequent finding, making Vp the most useful index to discriminate EMF patients.

Adult↗

PubMed1996

[Endomyocardial fibrosis with massive biventricular endocardial calcification].

Endomyocardial fibrosis is an endemic cardiac disease, characterized by the presence of fibrous tissue in the endocardium, eventually extending to the myocardium. Massive endocardial calcification of the left ventricle is a rare finding, with only a few cases reported in the literature. We reported a first case of biventricular massive endocardial calcification associated with endomyocardial fibrosis in a 22 year old woman.

Adult↗

PubMed1987

Surgical treatment of endomyocardial fibrosis.

Forty-six patients with endomyocardial fibrosis underwent endocardiectomy and replacement of tricuspid, mitral, or both atrioventricular valves between April, 1981, and October, 1984, at the Sree Chitra Tirunal Institute, Kerala State, India, which has a high incidence of the disease. Six patients were in New York Heart Association Functional Class III and 40 patients in Class IV. The operative mortality within 30 days of the procedure and late mortality during the first two years postoperation were 21.7% and 13%, respectively. Age under 15 years was a significant correlate of operative mortality (p = .05). Non-fatal thromboembolic episodes occurred in 6 patients during the two years of postoperative observation. The life table estimate of survival inclusive of operative mortality at two years was 67%. Despite high operative mortality, endocardiectomy with atrioventricular valve replacement is advisable for functionally disabled patients with endomyocardial fibrosis whose prognosis otherwise is dismal.

Adolescent↗

PubMed2001

Endomyocardial fibrosis in children.

We describe 10 children with endomyocardial fibrosis who underwent surgical treatment between 1978 and 1999. Seven were male and 3 female, with an age range from 4 to 15 years, having a mean age of 11 years. All were in the final stage of heart failure. Three had biventricular disease, 6 had involvement of the right ventricle alone, and one had endomyocardial fibrosis confined to the left ventricle. There were 3 deaths (30%) in the postoperative period due to low cardiac output. The 7 survivors were followed up for a period ranging from 12 to 168 months, with a mean of 72 months. Two late deaths have occurred resulting from heart failure and infectious endocarditis. Five (50%) children are still alive. Two required 3 reoperations for dysfunction of the inserted valvar prosthesis. One patient is in functional Class IV, and 4 are in Class II to III, despite intensive medical treatment. It is concluded that surgery for endomyocardial fibrosis is an essentially palliative procedure and, especially in children, the results of surgical treatment leave much to be desired.

Adolescent↗