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[14 cases of constrictive endocardial fibrosis (or endomyocardial fibrosis].

Constrictive endocardial fibrosis (CEF) or endomyocardial fibrosis (EMF) was studied on 14 files of this rather frequent disease in Ivory Coast. On the basis of the data derived from angiocardiography, catheterization and mechanograms, the clinical signs are interpreted better. Thus one is able to describe with accuracy some clinical forms, the commonest of which being a right heart restriction combined with left-sided signs. It seems important underline how useful the external pulse recordings might be useful for the diagnosis in view of their analogy with the pressure curves. In this disease with a severe course, bold surgical treatments provide some hope. On the contrary, its aetiology remains mysterious. Our best landmark being the link of CEP with tropical ecology, and in particular, in our cases, with the warm and damp forest ecology of some areas.

Adolescent

[Phonocardiographic and mechanographic study of 12 cases of constrictive endocardial fibrosis (or endomyocardial fibrosis)].

A phonomechanographic study of 12 patients with restrictive endomyocardial fibrosis (EMF) has highlighted the essential criteria for the diagnosis of this condition. The "ventricular" type of JVP, with a dip-plateau, is a reliable sign of right-sided EMF; this sign is more rarely of the tricuspid incompetence type. A similar picture emerges from recordings of hepatic pulsation, or other venous pulses. An apex-cardiogram with a dip-plateau is in favour of the diagnosis of left-sided EMF. The finding of a recordable infundibular impulse supports a left-sided EMF. Demonstration of an early-diastolic thrill, of endocardial origin, is an essential feature of this condition. It coincides with the rising phase of the dip, and was found in 10 of our 12 cases. There may also be murmurs arising from the pulmonary and atrio-ventricular valves. The characteristic feature of the carotid tracing is a prolonged pulse wave, with corresponding reduction of the haemodynamic quotient. These signs result from contraction of the potential filling space of the ventricle, from the restricted movement of the atrio-ventricular valves, and from a reduced blood flow.

Adolescent

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

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

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

[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

[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

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

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

Endomyocardial fibrosis and rheumatic heart disease in Mozambique.

The first five cases of endomyocardial fibrosis to be reported from Mozambique are described in an autopsy series over the years 1975-77, together with 85 cases of rheumatic heart disease in patients who died and were autopsied during the same period. Outside the central parts of the African continent, endomyocardial fibrosis does not seem as rare as previously thought. Observations on the age and sex of the patients correspond with what is already known about both diseases. The strikingly higher frequency of rheumatic heart disease discussed in this review is at variance with the findings of SHAPER et al. in Uganda over the years 1950-65 and poses problems as to the hypothesis of a common aetiological process in both diseases, i.e. a hypersensitivity mechanism acting on cardiac connective tissue.

Adolescent

The variety in endomyocardial fibrosis. A necropsy study.

In a necropsy series of 75 patients with endomyocardial fibrosis who died during the years 1967 to 1971 in Mulago Hospital, Kampala, the site of the lesions in the heart was studied alongside with the age, clinical history, geographical distribution, heart weight and concurrent diseases. Most frequently involved were the posterior cusp of the mitral valve, the left ventricular wall (upper-mid and/or apical region) and the right ventricular wall wheras othter localisations turned out to be relatively rare. Intracardial thrombi were also often described. Rheumatic heart disease was the most important concurrent cardiac disorder. The range and variety of the lesions is discussed together with relations to age, sex and geographical distribution of the patients. The results give support to Shaper's hypothesis concerning the aetiology of endomyocardial fibrosis; however many unanswered questions about the disease remain.

Adolescent

Pericardioperitoneal shunt for massive recurrent pericardial effusion in patients with endomyocardial fibrosis.

Massive recurrent pericardial effusion in patients with endomyocardial fibrosis presents a therapeutic challenge. Repeated pericardiocentesis and creation of a pericardial window is associated with significant morbidity and mortality in these critically ill patients. A new pericardioperitoneal shunt using a Holter valve and Harris catheter has been devised to solve some of these problems.

Adolescent

Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease.

Eleven patients with a restrictive cardiomyopathy are described. Seven of these had biventricular disease and in them the haemodynamic abnormality resembled that in constrictive pericarditis but the condition was distinguishable because of unequal involvement of the two ventricles, murmurs of atrioventricular valve regurgitation, or characteristic ventricular deformity on angiography. Two of these patients had eosinophilia with the clinical features of Löffer's endocarditis. In 4 patients the disease was apparently confined to the left ventricle; they were investigated on account of atypical chest pain, third heart sound, or abnormal electrocardiogram. Simultaneous measurements of left ventricular pressure and volume throughout diastole were made in 3 patients and showed rapid but abruptly curtailed left ventricular filling. Transvenous endomyocardial biopsy in 2 patients showed fibrous tissue with collagen and irregular elastic fibrils. Surgical biopsy in 3 patients excluded pericardial constriction but was diagnostically unhelpful because of failure to obtain endocardium. Necropsy in one patient showed that the heart had features indistinguishable from tropical endomyocardial fibrosis. It is suggested that the spectrum of ventricular abnormalities in these patients results from endomyocardial fibrosis of varying severity and probably of differing cause. This study has shown that "tropical" endomyocardial fibrosis may occur outside the tropics and suggests that eosinophilia may play a leading role or associated part in the genesis of some cases.

Adult

Endomyocardial fibrosis with calcification of the myocardium.

A case of endomyocardial fibrosis with intramyocardial calcification was characterized by symptoms of restrictive cardiomyopathy resembling constrictive pericarditis. Pathological findings were typical of those found in classical Davies' disease, originally encountered and described in Uganda.

Calcinosis

Endomyocardial fibrosis in a European woman and its successful surgical treatment.

A case of endomyocardial fibrosis of the right ventricle in a European woman is presented. The patient had no history of travel or residency outside of the United Kingdon, Echocardiography was helpful in defining the abnormality. Excision of the fibrotic tissue in the right ventricle and tricuspid valve replacement resulted in symptomatic relief. There was also coincidental absence of the right pulmonary artery.

Adolescent