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PubMed2001

[Endomyocardial fibrosis. Presentation as a right ventricular tumor].

Endomyocardial fibrosis is an uncommon disease in Spain, with the most frequent clinical feature being congestive heart failure syndrome. Complete atrioventricular block is exceptional. Two-dimensional echocardiography is a fundamental investigation achieving the diagnosis. Surgical treatment is recommended in symptomatic patients. We present a patient with heart failure secondary to endomyocardial fibrosis with complete atrioventricular block and an infrequent echocardiographic image of a polycystic mass in the free right ventricular wall.

Cardiomyopathies↗
PubMed1977

[Clinical aspects and course of endomyocardial fibrosis].

In 6 women with the hemodynamic, angiographic and histological findings of endomyocardial fibrosis the clinical course was followed for 30 months. All six cases showed the typical partial obliteration of one (4 patients) or both (2 patients) heart chambers. Left ventricular pressure-volume relations elicited decreased distensibility of the left ventricle. The functional state in 2 patients with severe mitral insufficiency was improved following successful mitral valve replacement. One patient with left ventricular, and 2 patients with biventricular, obliteration were given drug therapy and in one case recompensation lasting 2 years was obtained. One patient with partial obliteration of the left ventricle and severe biventricular failure died after an observation period of 4 years. In summary it can be said that (1) endomyocardial fibrosis in Switzerland is observed mainly in females; (2) the clinical picture is dominated by severe congestive heart failure in which the heart size is only slightly enlarged; (3) systolic performance is normal or only moderately depressed despite severe restriction to filling and/or av-valve insufficiency; (4) partial obliteration of the right or left ventricle may be detected by echocardiography; (5) rheumatic fever may occasionally be the cause of endomyocardial fibrosis; (6) mitral valve replacement is accompanied by appreciable improvement in the patient's condition.

Cardiomegaly↗
PubMedDate not supplied

Helminthiasis, the hypereosinophilic syndrome and endomyocardial fibrosis: some observations and an hypothesis.

It has been shown that chronic African endomyocardial fibrosis (E.m.f.) is most likely the burnt-out phase of parasite-induced hypereosinophilia. It has also been shown that African E.m.f. and Loffler's heart disease are pathologically identical. The mechanism by which these parasites and/or eosinophilia are associated with endomyocardial damage remains, however, unknown. The parasites which have been associated with induction of eosinophilia in E.m.f. include filariasis; trichinosis; ascariasis and hookworm and schistosomiasis. These parasites are known to produce neurologic, cardiac, pneumonic, hepatic and dermal damage during the migration of their larvae; at which time eosinophilia is usually most severe. The tissue damage induced by larval migration of these parasites appears comparable to findings seen in the hypereosinophilic syndrome. The evidence from our observations and this review suggests that the cardiac damage induced by larval migration, like the neurologic, pneumonic and dermal damage, is allergic in nature. Endomyocardial fibrosis has previously been shown to be an allergic heart disease. It appears reasonable to regard African endomyocardial fibrosis as representing the most intense, non-specific cardiac allergic reaction to helminthic larvae.

Adolescent↗
PubMedDate not supplied

Echocardiographic findings in endomyocardial fibrosis.

An 18-month-old infant diagnosed as having endomyocardial fibrosis by echocardiography is presented. Most patients with endomyocardial fibrosis reported in the literature are either older children or adults. To our knowledge, our patient was the youngest ever to have been reported. Echocardiographic studies showed obliteration of the left ventricular apex and increased echo reflectance at the left ventricular endocardium and subendocardium. The left atrium and right ventricle were significantly enlarged. Doppler echocardiography showed minimal mitral, but significant tricuspid regurgitation. In regard to the contribution of echocardiography in the diagnosis, we recommend this method for suspected cases. Contrary to the other patients reported, there was no thickening of the atrioventricular valves. Mitral valve insufficiency was related to the restriction of the ventricular filling rather than to valve involvement occurring with the disease.

Cineangiography↗
PubMed1971

Familial cases of endomyocardial fibrosis in Uganda.

This report describes nine cases of endomyocardial fibrosis occurring in four families. All patients came from Rwanda or South-western Uganda, and five had tropical splenomegaly syndrome as well. It seems likely that genetic as well as environmental factors are operative in the aetiology of endomyocardial fibrosis.

Adolescent↗
PubMed2002

Clinical meaning of ascites in patients with endomyocardial fibrosis.

OBJECTIVE: To evaluate the clinical meaning of ascites and the main features of patients with ascites and endomyocardial fibrosis. METHODS: We studied 166 patients with endomyocardial fibrosis (mean age 37 years, 114 women) treated over the last 20 years. Ventriculography findings, surgery or necropsy confirmed the diagnosis in all patients. Most patients belonged to New York Heart Association Functional Class III/IV (134, 83.7%). Eighty-one (50.6%) had biventricular, 28 (17.5%) had right ventricular, and 51 (31.8%) had left ventricular involvement. During follow-up, 56 patients died. RESULTS: Ascites was present in 67 (41.8%) patients, and right ventricular involvement was present in 59 (88%). In the comparison between patients with or without ascites, those with ascites had higher mortality (49.2% and 24.7%, respectively). Patients with ascites had a higher incidence of edema (95% vs. 43%), hepatomegaly (5.8cm vs. 4.1cm), mean right atrium pressure (19.3 vs. 12mmHg), and final right ventricle diastolic pressure (18.7 vs. 12.9mmHg). Also, patients with ascites had a longer history of illness (5.1 and 3.9 years, respectively) and had atrial fibrillation more frequently (44.7% vs. 30.1%). CONCLUSION: Ascites was observed in less than 50% of cases of endomyocardial fibrosis and was associated with greater involvement of the right ventricle and with a longer duration of the disease, thus being a characteristic of a worse prognosis.

Adolescent↗
PubMed1977

Pancarditis with valvulitis in endomyocardial fibrosis (=emf) and in human African trypanosomiasis (= hat). A comparative histological study of four Ugandan cases.

The study compares the histotopographical lesions of two cases of endomyocardial fibrosis with those of two hearts in sleeping sickness, all of them from Uganda. There was considerable overlapping of the fibrosed zones in endomyocardial fibrosis and the corresponding sites occupied by the chronic inflammatory process in trypanosomiasis. There was a tendency towards fibrosis in the hearts of trypanosomiasis while, in endomyocardial fibrosis, there were mild focal chronic cellular infiltrations. This overlapping and to some extent similarities would suggest that the two conditions may only differ in evolution in time and intensity and that they may be the result of the same inflammatory process. Stipulating a cardiac tropism for African trypanosomes, it is suggested that some African cases of endomyocardial fibrosis may be merely burnt-out lesions of trypanosomal pancarditis. Previous descriptions of carditis in human African and American trypanosomiasis are briefly reviewed, and the present findings are discussed in view of a possible trypanosomal aetiology in some African cardiomyopathies.

Endomyocardial Fibrosis↗
PubMed1996

Effect of protein deficient cassava diet on Cercopithecus aethiops hearts and its possible role in the aetiology and pathogenesis of endomyocardial fibrosis in man.

Despite the current hypotheses for its causation, the exact cause of endmyocardial fibrosis is unknown. However, endomyocardial fibrosis being a disease of the low socio-economic groups who feed on low protein high carbohydrate diets consisting exclusively of cassava in Uganda and the demonstration by the author of a bimodal age distribution among the female patients and monomodal pattern in the male patients, led the author to suspect protein deficiency and cassava as aetiological factors thereby attributing the first mode to the increased but unmet protein needs for childhood growth and the second mode to the increased but unmet protein needs for pregnancies and lactation in the 20-40 year age groups. Consequently a new hypothesis that "prolonged ingestion of tuber (cassava/tapioca) crops associated with extreme deprivation of protein causes EMF" was formulated. In order to verify this hypothesis, three Cercopithecus aethiops were fed on uncooked banana diet while another three were fed on uncooked cassava and hearts harvested for histology whenever the animal health deteriorated. Changes in the endomyocardium included cell vacuolation, interstitial fibrosis and endocardial thickening by the 130th day in the animals on cassava but the animals on bananas were free from such changes. By the 160th day, the former exhibited marked thickening of the endocardium, interstitial fibrosis, fibrous septa formation, pappillary muscle fibrosis as well as apical fibrosis of the left ventricle, which findings occur in the human disease. Calcification and inflammatory cells were absent. A repeat of the experimental feeding with cassava using a batch of five animals, one of which survived up to seven months revealed cardiac findings similar to those seen at 160 days. Thus, the pathogenetic process, hitherto obscure, begins with cardiac cell necrosis followed by fibrosis consequent upon the failure of cardiac cell repair due to protein deficiency caused by the protein free cassava diets since the animals on bananas, which also lacked protein did not develop similar changes. The low plasma amino acid profiles in EMF prone subjects, the poor blood supply and the great apical mechanical stress are incriminated for the severe apical lesions. This study shows that the disease can be experimentally induced in the monkey thereby validating the postulated hypothesis.

Adolescent↗
PubMed1980

Endomyocardial fibrosis: report of 6 patients and review of the surgical literature.

Six patients with endomyocardial fibrosis were treated by endocardium decortication and atrioventricular valve replacement. There were 5 female patients and 1 male patient ranging from 14 to 48 years old (mean, 30 years). Four patients had involvement of the right ventricle, 1 patient had involvement of the left ventricle, and 1 patient had biventricular disease. There was 1 operative death due to low cardiac output state (the patient with biventricular endomyocardial fibrosis), and there was 1 late noncardiac death. The surgical literature, which describes 19 previously reported cases, was reviewed. On the basis of the results of this series and those of the reported cases, it is concluded that surgical treatment of endomyocardial fibrosis is feasible and provides good clinical improvement.

Adolescent↗
PubMed2001

[Endomyocardial fibrosis in Behcet's disease: a case report of a pseudo-tumoral form].

Endomyocardial fibrosis is very rare in Behcet's disease. The authors report the case of a 28 year old patient with Behcet's disease complicated by a pseudo-tumoral right ventricular formation on echocardiography. This misleading appearance suggested the diagnosis of cardiac thrombus or tumour and led to a surgical approach which revealed a fibrous moderator band suggesting endomyocardial fibrosis, confirmed by antomopathological analysis. Besides the originality of this case and the unusual pseudo-tumoral presentation, the authors underline the difficulties of establishing the diagnosis, despite the advances of medical imaging. The pseudo-tumoral intra-cardiac lesion in a suggestive clinical context (Behcet's disease) should raise suspicion of the diagnosis of endomyocardial fibrosis.

Adult↗
PubMed1985

Benign course of endomyocardial fibrosis as a late complication of hypereosinophilic syndrome.

A case of Endomyocardial Fibrosis is reported, which retrospectively confirms the well known link between Endomyocardial Fibrosis and the Hypereosinophilic Syndrome in the temperature climate. We could trace back the history of this patient for 20 years. This suggests a benign form of endomyocardiopathy associated with mild cardiac restriction. 2-D apical echocardiography proved to be very useful as a non invasive tool for obtaining the correct diagnosis.

Endomyocardial Fibrosis↗
PubMed1997

Cerium levels are elevated in the serum of patients with endomyocardial fibrosis (EMF).

The geochemical hypothesis on endomyocardial fibrosis (EMF) links causation of the disease to increased levels of cerium in the heart. Since cardiac tissues are not easily accessible from patients, we explored whether cerium can be detected in the serum using neutron activation analysis (NAA). Cerium levels in serum of EMF patients were significantly elevated (p < 0.05) compared to controls.

Adolescent↗
PubMed1996

[Endomyocardial fibrosis of the right heart in Behçet disease].

Cardiac involvement is rare in Behçet's disease. Endomyocardial fibrosis of the right heart is exceptionally rare and is associated with right ventricular thrombosis. The authors report the case of a 27 year old man who died of a massive pulmonary embolism and who suffered from right-sided endomyocardial fibrosis with intraventricular thrombi. The diagnosis was made at autopsy. Behçet's disease was diagnosed on the finding of bipolar aphthous ulcers and skin hypersensitivity. The authors support previous workers in stating that Behçet's disease should be considered as a possible cause of endomyocardial fibrosis of the right heart.

Adult↗
PubMed2000

Usefulness of echocardiography and doppler echocardiography in endomyocardial fibrosis.

OBJECTIVE: Our goal was to demonstrate the usefulness of echocardiography and cardiac Doppler echocardiography (echo-Doppler) in the diagnosis of endomyocardial fibrosis, an unusual restrictive cardiomyopathy in Argentina. METHODS: Between 1980 and 1998, we studied 10 women (aged 27 to 58 years) with endomyocardial fibrosis confirmed by surgery and/or endomyocardial biopsy. Of the 10 cases of endomyocardial fibrothrombosis, 8 were biventricular and 2 were left ventricular. Six patients had only an echocardiographic study, and the last 4 patients (after 1987) had an echo-Doppler study also; 3 had a transesophageal echocardiography examination as well. Seven patients had grade III-IV dyspnea, 2 had an edematous-ascitic syndrome, and 1 had right heart failure at the first examination. Four patients died of heart failure and 1 of overimposed sepsis. Surgery was successful in 2 patients with the biventricular form of the disease. In one of them, fibrotic decortication was performed in both ventricles together with tricuspid and mitral replacement. In the other, the right side was not surgically treated because of its mild engagement. One patient was lost to follow-up, and 3 patients are awaiting surgery at this writing. RESULTS: In all 10 patients, echocardiography was the first diagnostic tool used. In M-mode echocardiography, the typical image showed the "square root" sign in the septum and posterior wall in addition to the "merlon" sign, characterized by a hypercontractile basal ventricle opposing an obliterated apex. In 2-dimensional echocardiography, inversion of the normal sized heart with obliterated ventricles and dilated atria were seen in the whole group. In 1 patient, the fibrous thrombus was limited to the apex of the right ventricle (Shaper's type 1) in a biventricular form, whereas in the left side of this patient and in the other 9 patients, the fibrous thrombus that initially occupied the apex engaged the posterior papillary muscle, pulling the posterior valve downward (Shaper's type 2) and generating tricuspid and/or mitral regurgitation that was always mild or moderate. The fibrous thrombus never altered the movement of the underlying myocardium. There were hypoechoic and hyperdense echoes inside the fibrotic material (the latter compatible with calcium), and in all 10 patients, different grades of pericardial effusion were found. Echo-Doppler showed the same minimal percentage of change in mitral and tricuspid velocities as found in healthy patients, which clearly differentiates endomyocardial fibrosis from constrictive pericarditis. Furthermore, a restrictive pattern was observed on both atrioventricular valves when both sides were engaged with a markedly short tricuspid deceleration time. Pulmonary veins showed a markedly diastolic D wave and a broad reversal A wave (the latter presented a low velocity when the wall of the left atrium was diseased) caused by an increased end-diastolic left ventricular pressure to the same extent throughout the respiratory cycle. Hepatic veins showed a markedly deep diastolic forward wave throughout the respiratory cycle and a marked reversal with inspiration. CONCLUSIONS: We showed (1) echocardiographic studies of a significant number of patients with this unusual disease, (2) the characteristic diagnostic signs in M-mode and 2-dimensional echocardiography, and (3) the common echo-Doppler patterns shared by all subjects studied with this technique.

Adult↗
PubMed1977

Endomyocardial fibrosis in Chandigarh area, India. A study of nine autopsies.

The clinical and autopsy data of nine cases of endomyocardial fibrosis encountered over a period of 11 years are presented. These account for nearly 24% of primary cardiomyopathies and 0.8% of cardiac diseases seen at autopsy. There were four cases with biventricular endomyocardial fibrosis, three with a predominantly left and two with a predominantly right ventricular affection. There was no correlation between the clinical data and the autopsy pattern of endomyocardial fibrosis; all of them presented as congestive cardiac failure.

Adolescent↗
PubMed1989

Determinants of survival in endomyocardial fibrosis.

This study describes the clinical course of 108 patients with endomyocardial fibrosis. There were 76 females and 32 males, with a mean age of 35 years. All patients underwent cardiac catheterization. The angiographic data show that 64 (59%) patients had biventricular involvement. From a clinical point of view, 91 (84%) were in New York Heart Association functional classes III and IV. According to the right and left ventricular intensity of fibrosis, the patients were classified as mild (40 [37%]), moderate (36 [33%]), or severe (32 [30%]) for the right ventricle and mild (19 [18%]), moderate (61 [56%]), or severe (28 [26%]) for the left ventricle. Tricuspid regurgitation was observed in 63 (58%) patients and mitral regurgitation in 65 (60%) patients. Fifty patients in functional classes III and IV underwent surgical treatment. Survival curves constructed according to functional classes showed better prognosis for those in classes I and II (p = 0.0452). Survival curves for patients in classes III and IV for clinical and surgical treatment showed a trend toward better results for the surgical group. Analysis of factors that may have influenced the course of endomyocardial fibrosis showed that biventricular involvement (moderate or severe), right ventricular fibrosis, and presence of tricuspid and mitral regurgitation were associated with greater mortality.

Adolescent↗
PubMed1983

Endomyocardial fibrosis. Preoperative diagnosis and surgical therapy.

The case of a Nigerian student with biventricular endomyocardial fibrosis is presented. Diagnosis was suggested by cardiac catheterization and histologically confirmed by a percutaneous endomyocardial biopsy. Successful surgical repair including mitral valve replacement, tricuspid valve reconstruction, and left ventricular endomyocardial resection was performed through a biatrial approach. The pathologic and surgical considerations are reviewed. This is one of the few cases of endomyocardial fibrosis reported from the United States and the first in which a percutaneous endomyocardial biopsy was used to provide a definitive preoperative histologic diagnosis.

Adult↗