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PubMed1990

Myocardial and endocardial involvement in tuberculous constrictive pericarditis: difficulty in biopsy distinction from endomyocardial fibrosis as a cause of restrictive heart disease.

Differentiation of constrictive pericarditis and endomyocardial fibrosis by the available diagnostic methods is difficult at present. With this in mind, we compared autopsy data in heart specimens from 8 patients of constrictive pericarditis of tuberculous origin and 6 patients with tropical endomyocardial fibrosis. Post-mortem endomyocardial biopsies were also performed from both the ventricles. Pericardial thickening and adhesions were present in all cases of constrictive pericarditis and in none of those with endomyocardial fibrosis. Interestingly, significant endocardial thickening was seen in four, and myonecrosis, lymphohistiocytic cellular infiltration and myofibrosis were observed in seven of the eight cases of constrictive pericarditis. This suggests that the tubercular insult to heart may result in involvement of all three layers with varying severity. The endomyocardial biopsy specimens from the affected areas revealed similar histopathological changes of endocardial thickening and focal myofibrosis with cellular infiltrates in patients with both constrictive pericarditis and endomyocardial fibrosis, making distinction of the two difficult. The hallmark of differentiation between these two clinical entities appears to be a diligent attempt at identification of the thickened pericardium.

Biopsy↗
PubMed1983

Enzymes of collagen synthesis and type III procollagen amino-propeptide in serum from Nigerians with chronic endomyocardial fibrosis.

The hallmark of cardiac pathology in chronic African endomyocardial fibrosis is the laying down of excess collagen in the endomyocardium. Preliminary observations on the assay of enzymes of collagen synthesis and type III procollagen aminopropeptide in chronic African endomyocardial fibrosis are reported. Serum immunoreactive prolyl hydroxylase protein was elevated in 4, serum galactosyl-hydroxylysyl glucosyl transferase activity was elevated in 5, and type III procollagen aminopropeptide was also elevated in 5 patients. Cardiac cirrhosis and endomyocardial fibrosis could be responsible for the elevated values in these patients. The relative contribution of fibrosis of the myocardium to these elevated values remains to be clarified.

Adolescent↗
PubMed1996

[Cardiac granulomas due to Schistosoma eggs and endomyocardial fibrosis].

Evidences of a possible association between endomyocardial fibrosis and schistosomiasis have been recently investigated. We describe the finding of cardiac schistosomal granulomas in a 14 year-old-girl presenting symptoms and signals of right side endomyocardial fibrosis. Refractory ascistis and progressive atrioventricular block were observed during follow-up. Endomyocardial biopsy and post mortem specimens showed inflammatory infiltrates, schistosomal granuloma and fibrosis.

Adolescent↗
PubMed1976

Endomyocardial fibrosis. Problems in differential diagnosis.

The clinical and angiographic findings in 5 consecutive patients with congestive cardiac failure are presented to illustrate the pitfalls in the clinical diagnosis of endomyocardial fibrosis. In one patient the clinical diagnosis was confirmed at angiography while another patient who had angiographic evidence of early right ventricular endomyocardial fibrosis was diagnosed clinically as mitral stenosis. In 2 patients the clinical diagnosis was erroneous, there being no evidence of endomyocardial fibrosis on angiography. The fifth patient, who had angiographic evidence of idiopathic cardiomegaly, was diagnosed clinically as either idiopathic cardiomegaly or advanced left ventricular endomyocardial fibrosis. In tropical countries, where endomyocardial fibrosis, rheumatic heart disease, and idiopathic cardiomegaly are common, accurate clinical diagnosis of endomyocardial fibrosis is often difficult and angiographic studies are essential for confirmation.

Adolescent↗
PubMed1970

The pathogenesis of endomyocardial fibrosis: the question of 5-hydroxytryptamine.

Thirty Nigerians with established endomyocardial fibrosis were studied. Basal serum 5-hydroxytryptamine levels were estimated on three different occasions at one-monthly intervals. Twentyfour hours before each estimation, dietary staples containing 5-hydroxytryptamine were excluded from the diet. Subsequently the patients were fed on a plantain diet and serum levels of 5-hydroxytryptamine were estimated at one- and three-hour intervals after plantain ingestion. 5-hydroxytryptamine was assayed on the fundal strip of rat's stomach. These values were compared with those obtained in an earlier study on healthy Nigerians. It was observed that, as in healthy Nigerians, no significant increase in serum 5-hydroxytryptamine levels occurred in these patients after plantain ingestion. The difference between endomyocardial fibrosis and carcinoid heart disease is underlined and it is emphasized that no correlation exists between the incidence of endomyocardial fibrosis and the high content of 5-hydroxytryptamine in the local dietary staples.

Diagnosis, Differential↗
PubMed1992

[The electrocardiogram in endomyocardial fibrosis].

PURPOSE: To detail the most common electrocardiographic findings on the endomyocardial fibrosis and to correlate with the ventricular form. METHODS: One hundred patients with endomyocardial fibrosis (68 female) with ages between 5 and 64 years old (mean 34 years). According to ventriculographic aspect the patients were divided in three groups: group I--11 patients with predominant right ventricular compromise; group II--58 patients with biventricular involvement, but not necessarily similar in intensity; group III--31 patients with predominant left ventricular compromise. RESULTS: On patients with predominant right ventricular compromise, the electrocardiographic pattern was of QRS complex of low voltage in the frontal plane, presence of incomplete right bundle block and QRS complex with low voltage and with qr or qs aspect in V1 contrasting with QRS complex of great amplitude on V2 and V3. Left anterior hemiblock, aspect of inactive area, and R waves of high voltage on left precordial leads were observed on patients with predominant left ventricular involvement. CONCLUSION: The electrocardiogram of endomyocardial fibrosis, in spite of inespecific, may help in the identification of ventricular involvement.

Adolescent↗
PubMed1993

[Endomyocardial fibrosis in children].

BACKGROUND: Recent advances in imaging techniques can facilitate the diagnosis of endomyocardial fibrosis. CASE REPORTS: Two cases of endomyocardial fibrosis were diagnosed in two Tunisian children, one aged 3 years and the other 12 years. Both were admitted with severe cardiac failure, predominantly of the right ventricule. Two dimensional echocardiography showed an apical amputation with echogenic material plus a thick, dense endocardial image, particularly at the level of the papillary muscles. The older child who also had cardiac catheterization, died 2 years later and the younger child died 4 years after diagnosis. Post mortem examination showed fibroelastic thickening of the endocardium and areas of fibrosis in the muscles of both children. CONCLUSIONS: The advantage of two-dimensional echocardiography over other imaging techniques is that it can confirm the diagnosis of endomyocardial fibrosis and indicate the extent and degree of fibrosis.

Child↗
PubMedDate not supplied

[Clinical features of constrictive endomyocardial fibrosis (author's transl)].

Constrictive endomyocardial fibrosis is very frequent in tropical areas with a hot and rainy climate. Young patients and specially male ones are generaly concerned. The onset is gradual and low-giving an adiastolic condition which may prevail on the right or the left heart. Some periods of more acute evolution may be observed. Death is caused by adiastolic disorders, thrombosis or arythmia. Aetiology is still unknown. A surgical treatment may be considered in some cases.

Adolescent↗
PubMed1996

Effects of cassava diet on Cercopithecus aethiops livers: a case for cassava as the cause of both tropical splenomegaly syndrome (TSS) and endomyocardial fibrosis (EMF).

The aetiology of endomyocardial fibrosis (EMF) and tropical splenomegaly syndrome (TSS) though speculative, was considered by the author to be the same or related since the two diseases may occur in the same individual and locality. Accordingly, when attempting to prove a hypothesis for the causation of EMF that prolonged ingestion of tuber (cassava/tapioca) associated with extreme deprivation of protein causes EMF; one group of three Cercopithecus aethiops was fed on uncooked cassava while a second group was fed with uncooked bananas and in addition to harvesting the hearts whenever the animal health deteriorated, livers were also harvested for histological changes. While hearts from the animals on cassava revealed changes seen in human EMF the livers from the same animals exhibited Kupffer cell hyperplasia and hypertrophy as well as sinusoidal lymphocytosis, features seen in human TSS thereby confirming that the aetiology of these two diseases is the same. However, the banana diet did not produce such changes.

Age Distribution↗
PubMed2005

Pitfalls in diagnosis and clinical, echocardiographic, and hemodynamic findings in endomyocardial fibrosis: a 25-year experience.

Endomyocardial fibrosis (EMF) is a fascinating disease entity of unknown etiology. It is prevalent in the tropical zone. Its essential features are the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of the inflow tract and apex of one or both ventricles. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary muscle involvement, and progressive reduction of the cavity of the involved ventricle leading to restriction in filling and atrial enlargement. This article will present 21 patients with EMF who were initially referred to our hospital from 1979 to 2004 with different diagnoses: rheumatic heart disease with mitral and or tricuspid regurgitation (n = 9), constrictive pericarditis (n = 6), restrictive cardiomyopathy (n = 1), hypertrophic cardiomyopathy apical type (n = 2), dilated cardiomyopathy (n = 2), and Ebstein malfunction of the tricuspid valve (n = 1). The clinical, echocardiographic, hemodynamic, and angiographic findings in these 21 patients are presented; echocardiographic findings lead to the right diagnosis. The presence of a small ventricle with obliteration of the apex and large atrium shown on two-dimensional echocardiography is highly suggestive of EMF.

Adolescent↗
PubMed1996

Geographical distribution of endomyocardial fibrosis in south Kerala.

BACKGROUND: Endomyocardial fibrosis (EMF) is a chronic heart disease confined to a few geographically specific locations within 15 degrees of the equator. Several aetiological hypotheses exist, among them filarial infection, eosinophilia, and toxic effect of the monazite element cerium from the soil. This study attempts to find out whether the pattern of distribution of EMF in south Kerala in India is consistent with the geochemical hypothesis. METHODS: From hospital records we identified all patients from south Kerala who had a confirmed diagnosis of EMF during the period 1978-1994. Our controls were patients from the southern districts diagnosed to have rheumatic heart disease (RHD) during the same period. We traced their residence address to the administrative subunit of taluk, and plotted the distribution of patients with EMF and RHD for each taluk in south Kerala. The taluks were then grouped into areas of high (> 4/100,000), medium (2.01-4/100,000), and low (< or = 2/100,000) density in each case. RESULTS: We identified an area of high density of EMF comprising four taluks near the coastline situated within the districts of Alapuzha, Kollam, and Pathanamthitta. Two coastal taluks in Kollam and Alapuzha districts are known areas of deposits of monazite elements in the state. Geographical distribution is not related to prevalence of filariasis and eosinophilia. CONCLUSION: Coexistence of high density of occurrence of EMF and deposits of monazite elements support the geochemical hypothesis.

Adolescent↗
PubMed1993

Endomyocardial fibrosis presenting as recurrent monomorphic ventricular tachycardia as the sole manifestation.

Endomyocardial fibrosis, a disease of unknown aetiology, is prevalent in the equatorial regions of the world. It is characterised by progressive fibrosis and endocardial thickening of one or both ventricular cavities, resulting in congestive heart failure and atrioventricular valvar regurgitation [1,2]. Although supraventricular arrhythmias in the presence of congestive heart failure have been reported [2,3] there is no published report of ventricular tachycardia being the sole manifestation of endomyocardial fibrosis. Here we report a biopsy-proven case of endomyocardial fibrosis where, in the absence of investigations, the patient was diagnosed as having idiopathic ventricular tachycardia for 2.5 years.

Adult↗
PubMed1986

A comparison of endomyocardial disease in the rat with endomyocardial fibrosis in man.

The features of 39 cases of spontaneous endomyocardial disease occurring in the rat heart are discussed and the condition is compared with endomyocardial fibrosis occurring in man. Rat endomyocardial disease is an age-related change characterized by subendocardial proliferation of spindle cells that may progress to a lesion histologically similar to fibrosarcoma. Human endomyocardial fibrosis, on the other hand, is not age-related and shows essentially fibrohyaline changes of low cellularity preceded by the occurrence of acid mucopolysaccharides in the subendocardial region; no evidence of malignant change has ever been reported.

Animals↗
PubMed1998

Long-term follow up of patients with endomyocardial fibrosis: effects of surgery.

AIMS: To determine the long-term outcome of patients with endomyocardial fibrosis and to compare echocardiographic and haemodynamic data before and after ventricular endocardial resection. PATIENTS: Seventeen patients (11 women and six men; mean age 35.5 years) diagnosed with endomyocardial fibrosis at the University Hospital in Zurich, Switzerland from 1971 to 1995. Twelve patients (70%) had partial obliteration of both ventricles and in five patients (30%) the fibrotic lesions were limited to the left ventricle. METHODS: Fourteen of the 17 patients had surgical resection: fibrosis was resected from both ventricles in five patients and from the left ventricle only in nine patients. Ten patients had mitral valve replacement and two had tricuspid valve replacement. Left ventricle endocardial resection was done without reconstruction or replacement of the atrioventricular valve in three patients. Preoperative and postoperative echocardiographic data were available for 11 patients and haemodynamic data for six patients. Patients were followed up for 0.4-19 years (mean 8.6). RESULTS: Preoperatively four patients were NYHA functional class IV and 10 were class III; postoperatively one patient was class III, seven class II, and six class I. Preoperatively, echocardiography showed obliteration of the left ventricular apex and inflow tract in all patients, which decreased or disappeared after surgery. Left ventricular end diastolic pressure decreased from 25 mm Hg before surgery to 14 mm Hg after successful resection of the fibrosis. Left ventricular and diastolic volume (normal 93 (17) ml/m2) increased from 65 ml/m2 to 97 ml/m2 (p < 0.05) after surgery. Ejection fraction was normal preoperatively (57%) and decreased slightly (52%) after surgery. One patient died five months after surgery from heart failure. Four surgically treated patients died during the follow up period: one each from systolic dysfunction, recurrence of endomyocardial fibrosis, pneumonia, and food poisoning. Overall survival was 65% at five years and 59% at 10 years; the survival rates of the operated patients was 72% and 68%, respectively. Only one of the medically treated patients survived longer than three years from diagnosis. CONCLUSIONS: Endomyocardial fibrosis is a rare disease in European countries and is found mainly in women. The clinical picture is characterised by severe congestive heart failure but heart size is only moderately increased. Systolic performance is normal or only slightly depressed despite severe restriction to filling, atrioventricular valve regurgitation or both. Partial obliteration of the right and/or left ventricle may be detected by echocardiography. Endocardial resection with atrioventricular valve replacement is the treatment of choice with appreciable postoperative improvement and 10 year survival of approximately 70%.

Adolescent↗
PubMed1996

Endomyocardial fibrosis: electron-beam CT features.

PURPOSE: To present morphologic and functional electron-beam computed tomographic (CT) features of endomyocardial fibrosis. MATERIALS AND METHODS: Twelve patients with histologic evidence of endomyocardial fibrosis and/or definitive echocardiographic and angiocardiographic findings were examined with electron-beam CT. RESULTS: Endomyocardial fibrosis was depicted as linear calcifications and/or a thin tissue band of low attenuation within the endomyocardium. In right ventricular involvement, CT depicted obliteration of the apex associated with extension along the free wall and/or papillary muscles of the tricuspid valve. In left ventricular involvement, a large amount of endocardial tissue led to severe obliteration within the inflow tract and/or of the apex. At ventricular volume analysis, restrictive patterns of the disease were determined. CONCLUSION: Electron-beam CT allows direct visualization of endomyocardial fibrosis and the resulting volumetric changes and enables distinction of the disease from constrictive pericarditis.

Adolescent↗
PubMed1992

Mitral valve repair in patients with endomyocardial fibrosis.

Between 1987 and 1990, 12 patients were operated on for endomyocardial fibrosis at our institution. Nine were treated by endocardectomy and mitral valve repair and constitute the material of this study. Ages ranged from 9 to 58 years (mean age, 32.5 years). Biventricular involvement was present in 3 cases, and 6 patients had predominantly left ventricular endomyocardial fibrosis. Six patients were in New York Heart Association class III/IV. Six patients had severe mitral insufficiency (3 to 4/4) and 3 patients had moderate mitral insufficiency (2/4). The operation consisted of left ventricular endocardectomy with complete detachment and mobilization of the posterior leaflet of the mitral valve in all cases. An autologous pericardial patch was used to reconstruct posterior leaflet continuity in 4 patients. In 7 patients a prosthetic mitral ring was used. In 3 patients right ventricular endocardectomy and tricuspid valve repair were also performed. All patients survived and none required early reoperation. Follow-up was complete and ranged from 9 to 38 months. No late deaths occurred, and 1 patient required mitral valve replacement for recurrent mitral regurgitation. Doppler echocardiographic studies were performed in 7 patients and revealed no or mild mitral insufficiency. In conclusion, mitral valve repair is safe and offers good early and late results in patients with endomyocardial fibrosis.

Adolescent↗
PubMed1983

Endomyocardial fibrosis in rats treated with N-nitrosomorpholine.

Endomyocardial fibrosis was observed after long lag periods in male Sprague-Dawley rats treated for 1-14 weeks with the carcinogen N-nitrosomorpholine. The fibrosis developed predominantly in the left ventricle. It occurred during 29-78 weeks after withdrawal of the carcinogen in 5% and 79-108 weeks after withdrawal in 20% of the experimental animals, but was never observed in controls of the same age. We suggest that endomyocardial fibrosis was induced by a direct effect of the carcinogen on the fibroblasts of the endomyocardium.

Animals↗