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PubMed1996

Endomyocardial fibrosis in Zimbabwe--how rare is it? A report of two cases.

In Africa endomyocardial fibrosis (EMF) is rare outside countries where it is endemic, such as Uganda, Mozambique and Ivory Coast. The only published case in Zimbabwe was in 1957. We describe two female patients aged 22 and 19 years who presented within seven months of each other with typical clinical, electrocardiographic and echocardiogaphic features of right ventricular EMF. We would like to suggest that EMF exists in Zimbabwe and needs to be considered in the differential diagnosis of patients presenting with suggestive clinical features.

Adult↗
PubMed1995

Successful reduction of endomyocardial fibrosis in a patient with idiopathic hypereosinophilic syndrome. A case report.

A case of endomyocardial fibrosis in a patient with idiopathic hypereosinophilic syndrome is reported and discussed. The authors draw attention to the importance of both echocardiography (two-dimensional and Doppler) and nuclear magnetic resonance in the detection of cardiac involvement due to this rare pathology. Moreover, these imaging techniques appear to be valuable in the evaluation of effects of medical treatment. Therapy with corticosteroids alone has shown no reliable effectiveness in reducing the absolute eosinophil count. A combined immunosuppressive treatment with use of hydroxyurea is required.

Dipyridamole↗
PubMed1983

Coronary angiographic features in endomyocardial fibrosis.

Selective coronary cineangiography was performed in 24 patients with angiographic features suggestive of endomyocardial fibrosis (EMF). 8 patients had right ventricular disease, 2 had left ventricular disease and 14 had biventricular disease. The abnormal features found in the coronary arteries of these patients were: (a) vascular blush or neovascularity in 54.2% of patients and (b) localized ventricular filling from the region of the blush in 16.7%. Generalized tortuosity of the coronary arteries, considered to be a nonspecific change associated with ventricular enlargement and hypertrophy, was seen in 50%. This study lends support to one of the hypotheses which implies the role of mural thrombus in the evolution of the pathological lesion of EMF.

Adolescent↗
PubMed2004

Tricuspid aseptic endocarditis revealing right endomyocardial fibrosis during an unrecognized Behçet's disease. A case report.

INTRODUCTION: Aseptic endocarditis or/and endomyocardial fibrosis are rarely reported in Behçet's disease. OBSERVATION: We report on a case of a 21-year-old man living in Algeria, revealed by verrucous tricuspid valvulitis extending to the ventricular endomyocardium and complicated with right heart failure, initially misdiagnosed and treated as infective endocarditis occurring on rheumatic cardiac after-effects. DISCUSSION; We discuss the lack of specificity of Jones criteria and emphasize the need to include cardiac involvement in Behçet's disease in the differential diagnosis of rheumatic fever carditis. This message is notably important in some countries where the prevalence of these two entities are among the highest in the world.

Adult↗
PubMed1983

Early angiographic features of right ventricular endomyocardial fibrosis.

Right ventricular (RV) angiograms were done in 45 patients with left ventricular (LV) endomyocardial fibrosis (EMF). 34 patients had angiographic features compatible with the diagnosis of RV and LV EMF. 11 patients showed the angiographic features of EMF on the left side, but did not show the classical angiographic features on the right side. The RV angiograms of these 11 patients showed a disturbance of the normal parenchymal pattern along the septal margin, irregular small filling defects near the apex and the septal region and minimal tricuspid regurgitation. Biopsies from the RV taken from 2 of these patients during operation for mitral valve replacement had histological features compatible with the diagnosis of EMF on the right side also. This study demonstrates that the early angiographic features of RV EMF differ from those of well-established cases already described in the literature, and that it is possible to spot RV EMF well before the conventional features appear in RV angiograms.

Adult↗
PubMed1982

Skeletal muscle involvement in tropical endomyocardial fibrosis.

Evidence for neuromuscular dysfunction was sought in 20 consecutive, angiographically proven cases of tropical endomyocardial fibrosis (Group I). Statistical comparison was made with equal number of age, sex and weight-matched normal healthy volunteers (Group II), as well as similarly matched cases of consecutive heart failure (Group III). A myopathic pattern in the EMG was found in 85% of Group I and 65% of Group III patients. However, the severity and global extent of the EMG changes was much more marked in Group I than in Group III. The summed motor unit potential duration was reduced by 20.1% in Group I and by 9.5% in Group III. Corresponding figures for summed motor unit potential amplitude were 27.9% and 15.4%. the summed frequency of polyphasic potentials was significant in Group I and in Group III. Involvement of 3 or 4 muscles in the same patient was found in 80% of Group I, n 10% of Group III. The upper limb muscles were affected in 80% of Group I and 50% of Group III. The motor nerve conduction velocity and distal latency time were normal in all the patients. It is suggested therefore that the myopathic EMG changes in Group I patients may be related to the disease process itself, rather than a reflection of long standing CHF alone. Light-microscopic study of the quadriceps muscles in 8 patients of Group I did not reveal any abnormality.

Adolescent↗
PubMed1998

Atrial fibrillation in endomyocardial fibrosis is a marker of worse prognosis.

We studied the incidence of AF in patients with endomyocardial fibrosis (EMF) and its influence on prognosis and associated clinical events. One hundred and sixty consecutive patients with EMF were followed for a mean period of 4 years. Their mean age was 39.7 years. There were 114 women. During follow-up there were 56 deaths. Eighty-eight patients (55%) were submitted to surgical intervention. AF was observed in 58 cases (36.2%). The presence of AF was associated with a greater prevalence of dyspnea, peripheral edema, hepatomegaly, lower left ventricular ejection fraction, lower right ventricular systolic pressure (37.8 vs 45.6 mmHg, P=0.0392), and greater incidence of tricuspid regurgitation (86.0 vs 63.2%, P=0.004). AF was more frequent among patients in whom the disease involved the right ventricle, particularly those with intense fibrosis. Overall, patients with AF had a higher mortality rate than those who did not have AF (43.1 vs 30.3%, P=0.0195), but among those submitted to surgery, AF did not have an impact on survival. In conclusion, AF is frequent among patients with EMF. It is more prevalent among patients with right ventricular involvement and its presence is associated with a greater incidence of heart failure. AF is associated with worse prognosis, but surgery potentially reverses this bad evolution.

Adult↗
PubMed1985

[Anatomo-clinical forms of left endomyocardial fibrosis. Study of 22 surgically treated cases].

The authors studied 22 patients with operated left sided endomyocardial fibrosis (EMF). Twenty patients had pure left sided and 2 bilateral (mainly right sided) EMF. The mean age of the patients was 14 years (range 7-51 years). Three clinico-pathological forms of the disease were identified: obliterating, diffuse and limited. The mechanism of associated mitral regurgitation was papillary muscle and/or posterior leaflet involvement. The anterior mitral leaflet was not affected in any of these cases. Echocardiographic diagnosis was possible in 18 cases by M mode recording showing M-shaped septal wall motion. The diagnosis was made in 5 out of 13 patients by 2D echocardiography. This method was satisfactory in the obliterating form of the disease but direct visualisation of the fibrosis in the diffuse and limited forms was very difficult. Catheter studies showed severe pulmonary hypertension in all cases. The presence of a dip-plateau pressure recording was inconstant and was only observed in the obliterating and diffuse forms of EMF. Angiocardiography was characteristic in the obliterating and diffuse forms and enabled an accurate anatomical diagnosis before surgery. The diagnosis of the limited form was difficult and could only be suspected in cases of mitral regurgitation associated with right ventricular EMF or in the presence of parietal recesses. This surgical series does not include cases of EMF limited to the ventricular apex without mitral regurgitation or adiastole, treated medically.

Adolescent↗
PubMed1998

Alterations in collagen metabolism and increased fibroproliferation in the heart in cerium-treated rats: implications for the pathogenesis of endomyocardial fibrosis.

Cerium (Ce), a rare earth element, has been postulated to play a role in the pathogenesis of tropical endomyocardial fibrosis (EMF). Investigations carried out recently in pursuance of the postulation furnished histological evidence of EMF and increased cardiac collagen content in rats on prolonged administration of Ce. The present study was undertaken to understand the molecular basis of myocardial injury and fibrosis produced by the element. This article presents evidence of increased lipid peroxidation and elevated rates of fibroblast proliferation and collagen deposition in the heart in Ce-treated rats. It is suggested that the element may trigger a wound-healing response in the cardiac tissue leading to cardiac fibrosis.

Animals↗
PubMedDate not supplied

Endomyocardial fibrosis (Davies disease) coincidental with systemic lupus erythematosus.

This is the case of a 27 years-old woman with signs and symptoms of severe untreatable congestive heart failure, anemia, gingival mucosa ulcers, photosensitivity and alopecia. The electrocardiographic, echocardiographic, angiographic and hemodynamic data oriented the diagnosis of restrictive cardiomyopathy, mitral insufficiency secondary to mitral prolapse and bi-atrial dilation. The histologic study of the endomyocardial biopsy, performed during catheterization, showed signs of endomyocardial fibrosis, and immunological analysis was compatible with systemic lupus erythematosus. As far as we know, this is the first case of endomyocardial fibrosis (Davies disease) associated with systemic lupus erythematosus published in the medical literature. The etiology of Davies disease remains unrevealed and its association with systemic lupus erythematosus suggest a probable autoimmune origin.

Adult↗
PubMed1990

Right ventricular endomyocardial fibrosis. Diagnosis and management.

The clinical course, noninvasive and invasive diagnostic findings in 4 patients (pt) with endomyocardial fibrosis are reported. All patients (16-50 years) were in functional class III-IV (NYHA). Central venous pressure with large a- and v-waves was elevated in all; liver enlargement and peripheral edema were also noticed in all pts, and ascites in 3. An apical 2/6 systolic murmur was present in 2, and a right parasternal pansystolic murmur 2-4/6 with positive Carvallo's sign in all pts. ECG was non-specific and chest x ray showed right atrial enlargement in every case. A right-sided diastolic plateau which was higher than left ventricular end-diastolic pressure was present in all pts. Echo-, angiocardiography and computed tomography of the heart revealed obliteration of the right ventricular cavity, predominantly localized at the apex. Two pts who underwent endocardial resection and tricuspid valve replacement are alive and well after 9 and 8 years, respectively. One pt died early in the postoperative period and one died waiting for surgical therapy. In conclusion, echo-, angiocardiography and computed tomographic findings are diagnostic. A satisfactory differentiation from other cardiac disorders with restrictive hemodynamics and right-sided heart failure is possible.

Adolescent↗
PubMed1985

The surgical treatment of endomyocardial fibrosis: results in 55 patients.

Over a 5 year period we have operated on 55 patients with endomyocardial fibrosis (EMF). The patients were from 4 to 56 years old (mean 13) and they were usually in poor condition at the time of surgery, with 47% in class III, and 24% in class IV with respect to cardiac function. They presented with right heart restrictive diastolic filling pattern (n = 17), mitral insufficiency (n = 18), or a combined syndrome (n = 20). All patients were operated upon during cardiopulmonary bypass and were submitted to a combination of the following procedures: endocardiectomy (30 right ventricular, 28 left ventricular), valvular replacement (21 tricuspid valve, 27 mitral valve), and/or valvuloplasty (10 mitral, five tricuspid). Nine patients, all with left ventricular or bilateral EMF, died in the postoperative period (16%), mainly of low cardiac output. There were five late deaths (three valve related). All other patients were functionally improved at the mean follow-up of 32 months. Twenty-two underwent a late catheterization study. We conclude the following from our experience: EMF is a very severe disease that is seen mainly in childhood and adolescence, carries a poor spontaneous prognosis, and is frequently seen in tropical countries. Surgical treatment is beneficial, but surgical mortality is high. Our technical modifications have totally eliminated the occurrence of postoperative complete heart block. A conservative valvular procedure has been shown to be possible even in some patients with severe mitral insufficiency and left ventricular EMF. The late objective improvement is better in those with left ventricular EMF than in those with right ventricular EMF.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1982

Clinical feminization and serum testosterone levels in male patients with chronic African endomyocardial fibrosis.

Thirteen out of 19 male patients aged sixteen years and above who had clinically diagnosed chronic African endomyocardial fibrosis (E.M.F.) had some clinical evidence of feminization. Serum was obtained from ten patients and assayed together with serum of ten age- and sex-matched normal controls for testosterone. The serum testosterone level in the 10 E.M.F. patients was uniformly below the lowest normal level, and the mean serum level for E.M.F. was significantly lower than the mean for the controls. Persistent and severe hepatic congestion in E.M.F. may lead to feminization long before cardiac cirrhosis occurs.

Adolescent↗