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PubMed1999

Poverty and eosinophilia are risk factors for endomyocardial fibrosis (EMF) in Uganda.

OBJECTIVE: To determine the relative risks of socio-demographic, dietary, and environmental factors for endomyocardial fibrosis (EMF) in Uganda. METHOD: Unmatched case control study in Mulago Hospital, Kampala. Cases (n = 61) were sequential patients hospitalized with an echocardiographic diagnosis of EMF from June 1995 to March 1996. Controls (n = 120) were concurrent patients with other forms of heart disease (heart controls, n = 59) and subjects admitted for trauma or elective surgery (hospital controls, n = 61). All consenting subjects answered a structured questionnaire administered by trained interviewers. Complete blood counts, malaria films and stool examination for ova and parasites were performed. Questionnaires elicited information on home address, economic circumstances, variables concerned with environmental exposures and usual diet before becoming ill. RESULTS: After adjustment for age and sex, cases were significantly more likely than controls to have Rwanda/Burundi ethnic origins (P = 0.008). Compared with controls, cases had a lower level of education (P < 0.001 for heart controls and P = 0.07 for hospital controls), were more likely to be peasants (P < 0.001), and to come from Luwero or Mukono Districts (P = 0.003). After further adjustment for peasant occupation, cases were more likely than controls to walk barefoot (P = 0.015), consume cassava as their staple food (P < 0.001) and to lack fish or meat in dietary sauces (P = 0.02). Cases were more likely to exhibit absolute eosinophilia (P = 0.006). The effect of cassava diet was more marked in the younger age group, while the effect of eosinophilia was greater in adults. Socio-economic disadvantage is a risk for EMF. Absolute eosinophilia is a putative cause of EMF, a finding not explained by parasitism. CONCLUSION: Data indicate that relative poverty and environmental factors triggering eosinophilia appear to act in a geographically restricted region of Uganda in the aetiology of EMF.

Adolescent↗
PubMed1990

[The hypereosinophilic syndrome in a child with endomyocardial fibrosis treated surgically].

The authors describe the case-history of a 12-year-old patient with hypereosinophil syndrome, endomyocardial fibrosis of the left ventricle and dominating mitral valve insufficiency. By correction of the insufficiency, using a Carpentier ring, the authors achieved substantial improvement of the haemodynamics. One year after operation the patient is cardially compensated.

Child↗
PubMed1984

[Conservative valve surgery in endomyocardial fibrosis. Apropos of 8 cases among 46 surgically treated patients].

The authors report their experience of conservative valvular surgery in endomyocardial fibrosis (EMF) (8 cases among 46 surgical cases). These patients were divided into 3 groups: Group I: bilateral EMF predominating on the left side with associated tricuspid involvement (n: 3). Group II: bilateral EMF predominating on the right side with moderate associated mitral involvement (n: 3). Group III: unilateral EMF with massive mitral insufficiency, (n: 2). At surgery, on the side of the conserved valve, the patients had a transvalvular endocardectomy (n: 3) and a valvuloplasty either tricuspid (n: 3) or mitral (n: 5). The results of the valvuloplasty were satisfactory. Two patients died in the post-operative period. Their death was unrelated with the conservative procedure. The authors study the literature concerning the cases of valvular conservative surgery in EMF (n: 4) and define the conditions under which this surgery can be done. They report 2 original cases of pure left sided limited EMF with massive mitral insufficiency successfully treated by a valvuloplasty.

Adolescent↗
PubMed1998

Helminth associated hypereosinophilia and tropical endomyocardial fibrosis (EMF) in Nigeria.

There should be a recognisable trend between the incidence of hypereosinophilia and the duration of tropical endomyocardial fibrosis (EMF), if the hypothesis, that EMF is the burnt out phase of eosinophil associated heart disease, is correct. We tested this hypothesis in a prospective study of 89 consecutive EMF cases over an 18 year period at two Nigerian locations (Ife in South West and Calabar in South East). We carefully dated the duration of EMF symptoms at first presentation and screened for hypereosinophilia (eosinophilia > or = 1500/mm3), and their causes. When no cause was identified for hypereosinophilia we gave a therapeutic trial with diethylcarbamazine to the patients. An eosinophil count > or = 1000/mm3 was recorded in 80% of 24 cases seen within 6 months; 55% of 46 cases seen 0-24 months; 54% of 18 cases seen 25-48 months and 21% of 25 cases seen more than 49 months of onset of symptoms: while the respective distribution of eosinophil count > or = 1500/mm3 for similar periods were 66, 44, 27 and 21%. This reflects a highly significant (P < 0.001) inverse relationship between hypereosinophilia and the duration of EMF and strengthens the concept that EMF (without eosinophilia) represents the late stage of eosinophilic heart disease. The localisation of endemic EMF to the low-lying tropical rain forest Zone and its predominant occurrence among rural dwellers and farmers suggest a vector borne etiologic agent. Microfilaria was the most likely cause of hypereosinophilia in the cases presented.

Adolescent↗
PubMed1990

Distribution of antibodies against Coxsackie B viruses, arboviruses and Toxoplasma gondii among patients with endomyocardial fibrosis (EMF) compared with normal subjects from EMF endemic and non-endemic zones of Nigeria.

The sera of eight endomyocardial fibrosis (EMF) subjects, 11 siblings of one of them and 16 normal children matched with the EMF patients for age, sex and socio-economic status from Ogunmakin and Shao/Oloru communities (eight each), situated in EMF-endemic and non-endemic areas of Nigeria respectively, were examined for the presence of antibodies against Coxsackie viruses B1-6, 16 arboviruses and Toxoplasma gondii. Sera from 36 other randomly selected normal children from Ogunmakin and 26 other randomly selected children from Shao/Oloru were also tested for the presence of antibodies against Toxoplasma gondii and the 16 arboviruses. None of the eight EMF subjects nor the 11 siblings of one of them had antibodies against any of the Coxsackie viruses B1-6 in their sera. Two of the 16 matched control subjects, one from each community, had positive antibodies, at equivocal titres against Coxsackie B1 (Ogunmakin) and B4 (Shao/Oloru). There was no significant difference in the distribution of antibody titres to the arboviruses between the EMF patients and matched controls. Normal children from the Shao/Oloru community had higher percentage antibody reactions and higher titres to the arboviruses compared with the children from Ogunmakin. All the eight EMF patients had high antibody titres against Toxoplasma gondii. Seven (87.5%) of the matched controls from Ogunmakin were sero-positive for Toxoplasma gondii compared with three (37.5%) of the matched controls from Shao/Oloru. Of the 36 normal children from Ogunmakin, 32 (88.9%) were sero-positive compared with 11 (42.3%) of the 26 normal children from Shao/Oloru. Four (36.4%) of the 11 siblings of one of the EMF patients had weak sero-positivity. It is therefore concluded that further studies are needed to clarify the role, if any, of Toxoplasma gondii in EMF.

Adolescent↗
PubMedDate not supplied

Accumulation of glycosaminoglycans associated with hypomagnesaemia in endomyocardial fibrosis in Kerala: possible involvement of dietary factors.

Nutritional factors, mainly low protein intakes have been implicated in the pathogenesis of endomyocardial fibrosis (EMF), the incidence of which is high in Kerala. As there is only marginal protein deficiency in the Kerala population, this may not be a causative factor. Studies have revealed low levels of magnesium (Mg) in the serum of these patients and high concentration of glycosaminoglycans. Accumulation of glycosaminoglycan-associated Mg deficiency is observed in the serum of EMF patients. The heart tissue from autopsy samples of EMF patients also showed accumulation of glycosaminoglycans.

Adult↗
PubMed2000

Endomyocardial fibrosis in China.

PURPOSE: To introduce the epidemical, pathological, and clinical characteristics as well as the diagnostic and therapeutical experiences of endomyocardial fibrosis (EMF) in China. DATA SOURCES: A CMBdisc search was done of the Chinese-language literature published from January 1983 through June 1997 about EMF and/or restrictive cardiomyopathy. A manual search was then done for other contributions, including abstracts, between January 1965 and June 1997. RESULTS: Eighty-seven Chinese cases of EMF were collected in this paper. There were 49 men and 38 women, with a mean age of 28 +/- 13 years (range, 8 to 68 years). The distribution of the cases is mainly in the south of China. Combined right and left ventricular disease occurs in 48 percent of cases, with pure right ventricular involvement occurring in 12 percent and pure left ventricular involvement in the remaining 10 percent of patients who are examined postmortem. The diagnosis of EMF was confirmed in 21 cases at autopsy, and in 66 cases by echocardiography, angiocardiography, and/or endomyocardial biopsy which showed the characteristic changes. Clinically, right-sided disease is the commonest variety. Endocardiectomy and tricuspid (n - 7) or mitral (n - 1) valves replacement have been performed in 8 patients. There were 2 operative deaths. Six patients had a satisfactory recovery postoperatively and living well in the follow-up duration. CONCLUSION: EMF has been diagnosed clinically and confirmed at necropsy in a number of cases in the south of China. The etiology, incidence and epidemiology are still unknown. The pathological and clinical features are similar to those in tropical areas, but right ventricular involvement is the commonest type in our country.

China↗
PubMed1982

A comparison of the clinical, haemodynamic and angiographic features in right ventricular endomyocardial fibrosis and Ebstein's anomaly of the tricuspid valve.

The clinical, haemodynamic and angiographic features of 18 patients with right ventricular endomyocardial fibrosis (RVEMF) and 8 patients with Ebstein's anomaly of the tricuspid valve (EATV) have been compared. Diagnosis was confirmed by selective angiography. The position of the tricuspid annulus was identified from selective right ventricular angiograms and confirmed by selective right coronary angiography. In 83% of RVEMF patients the tricuspid annulus was displaced to the left of the spine. A false impression of displacement of the tricuspid leaflet can thus be created. However, a tricuspid leaflet displaced away from the tricuspid annulus was found only in patients with EATV. A considerable overlap exists between the wide spectrum of clinical presentations of the two conditions. Helpful distinguishing features that favour EATV were, the presence of a scratchy diastolic murmur and polyphasic QRS complexes in the ECG. Atrial fibrillation in the ECG, and myocardial calcification or pericardial effusion, whenever present, favour RVEMF.

Adolescent↗
PubMed1986

Unusual echocardiographic appearance of intracardiac thrombi in a patient with endomyocardial fibrosis.

A five year old girl presented with a four month history of recurrent heart failure, which subsequently proved to be caused by endomyocardial fibrosis. There was no evidence of valvar disease. Echocardiography showed several echogenic masses with echolucent centres within the cardiac cavity. Histological examination showed that these masses were partly organised thrombus.

Child, Preschool↗
PubMedDate not supplied

Echocardiographic profile of endomyocardial fibrosis in Tanzania, East Africa.

OBJECTIVES: To demonstrate and evaluate the usefulness of two dimensional and Doppler echocardiography in the diagnosis of endomyocardial fibrosis (EMF) in countries with poor resources. Also to evaluate the clinical assessment as a predictor of echocardiographically proven EMF. DESIGN: Descriptive hospital based study. SETTING: Muhimbili National Hospital, Dar es Salaam, Tanzania. SUBJECTS: 39 patients (27 male and 12 female, mean age 13.5 years) attending our Cardiac Clinic were investigated to determine the extent to which specific features could be diagnosed by transthoracic echocardiography. MAIN OUTCOME MEASURES: Identifying and characterizing echocardiographic features specific for diagnosing EMF non-invasively. RESULTS: Only eight (21%) patients had a correct clinical diagnosis, leaving 79% of the patients at risk of being misdiagnosed and hence wrongly receiving expensive treatment. The majority of the patients (69.2%) presented with signs of elevated systemic venous pressure due to right ventricular EMF. CONCLUSION: We have demonstrated that echocardiography remains a fundamental investigation in the least developed countries in achieving the correct diagnosis.

Adolescent↗
PubMed1988

Surgical treatment of biventricular endomyocardial fibrosis. A case report.

The clinical, fono-mechano-echocardiographic, hemodynamic and angiographic findings before and after surgery of a patient who developed bilateral extensive endomyocardial fibrosis probably secondary to filariasis, are described. The patient was surgically treated by endocardial decortication and insertion of a mitral and tricuspid Ionescu Shiley low profile valve, which was followed by a marked subjective and objective improvement during a follow-up period of 42 months.

Angiography↗
PubMed1999

Neovascularity related to mural thrombus in endomyocardial fibrosis.

We report a 30-year old, previously healthy, Ghanese woman admitted with sudden onset of dyspnoe, hemoptoe and right-sided chest pain due to endomyocardial fibrosis with secondary pulmonary emboli Coronary angiography revealed a myocardial "blush". This finding may focus attention to the presence of mural thrombus that may have diagnostic and therapeutic consequences.

Adult↗
PubMed1980

Surgical treatment of endomyocardial fibrosis with preservation of mitral valve.

A 48-year-old Kenyan African, who presented with a history of coronary and cerebral embolism, was found to have endomyocardial fibrosis of the left ventricle. It proved possible to remove all diseased tissue at operation, and at the same time to preserve the mitral valve. It is important to inspect the mitral valve from both the atrial and ventricular aspects so that the valve can be spared, if it is not involved in the disease process. The choice of transatrial of transventricular surgical approach for the removal of pathological tissue may depend on whether or not the mitral valve requires replacement.

Endomyocardial Fibrosis↗
PubMed1991

[Mitral valvuloplasty for endomyocardial fibrosis in a child with acute leukemia and hypereosinophilia].

The authors report the case of a child with acute lymphoblastic leukaemia and hypereosinophilia complicated by left sided endomyocardial fibrosis. Despite the need for urgent treatment and severe mitral valve disease, a complex mitral valvuloplasty was performed, consisting of mobilisation and reconstruction of the posterior leaflet, burying the chordae with plicature of the papillary muscle of the anterior leaflet associated with an annuloplasty. Decortication was performed by a transvalvular approach. The technique of mobilisation-reconstruction of the posterior mitral leaflet with a pericardial patch should widen the indication of conservative surgery in this condition, in the presence of dominant mitral regurgitation in the child.

Child↗
PubMed1981

Adriamycin cardiotoxicity: report of an unusual case with features resembling endomyocardial fibrosis.

We report on a case of adriamycin cardiotoxicity occurring in a five-year-old boy treated for rhabdomyosarcoma. In addition to the usual features of myofibrillary degeneration associated with adriamycin, extreme endocardial fibrosis and mural thrombosis affecting the apical segments of both ventricles but particularly the left ventricle was seen at necropsy. The changes resembled classical endomyocardial fibrosis.

Cardiomyopathies↗
PubMed2002

Churg-Strauss syndrome with critical endomyocardial fibrosis: 10 year survival after combined surgical and medical management.

A case is presented of the Churg-Strauss syndrome with hypereosinophilia and severe cardiac involvement, namely biventricular endomyocardial fibrosis and gross encroachment of the right ventricular cavity. The clinical picture was similar to Loeffler's syndrome and the idiopathic hypereosinophilic syndrome. Combined aggressive surgical and medical management led to full recovery and survival at 10 years. The good long term outcome is attributed to strict control of peripheral eosinophil count by oral corticosteroids. This case illustrates the damaging effects of hypereosinophilia on the heart.

Churg-Strauss Syndrome↗