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[Restrictive myocardiopathy caused by endomyocardial fibrosis].

Endomyocardial fibrosis, an uncommon condition, is the most important cause of restrictive cardiomyopathy. Etiology of endomyocardial fibrosis is unknown, but evidence continues to accumulate that a close connection exists between eosinophilia and endomyocardial disease. Patients with this condition respond poorly to medical treatment, and survival is only for a few months or years when late stage heart disease is present. Surgery is nowadays recommended in these cases. A marked eosinophilia was found in a 58 years old woman that was admitted to the hospital because of a severe congestive cardiac insufficiency. Two-dimensional echocardiographic study and typical angiographic appearance suggested the presence of endomyocardial fibrosis. Endocardectomy and mitral valvular replacement were performed.

Cardiomyopathy, Restrictive↗

[Heart failure in a Ghanese woman due to endomyocardial fibrosis].

Endomyocardial fibrosis was diagnosed in a 30-year-old Ghanese woman suffering from heart failure and intracardial thrombi. Endomyocardial fibrosis is an endemic disease in tropical countries with a high mortality rate. Since the aetiology of this disease is still unknown, patients can only be treated symptomatically. Endocardial and myocardial damage give rise to reactive fibrosis, subendocardial infarction and thrombus formation, resulting in heart failure and thromboembolic processes.

Adult↗

Total cavopulmonary connection for right ventricular endomyocardial fibrosis.

Endomyocardial fibrosis is an endemic problem in tropical countries and is characterised by ventricular cavity obliteration, decreased ventricular compliance, and atrioventricular valve regurgitation. We report on a patient with right ventricular endomyocardial fibrosis resulting in obliteration of the cavity and tricuspid regurgitation treated successfully by total cavopulmonary connection and exclusion of the right ventricle.

Adult↗

Epidemiology aspects of endomyocardial fibrosis.

Endomyocardial fibrosis (EMF) is a specific clinicopathological entity. It occurs sporadically throughout the world but is endemic in parts of Africa, India and South America. In sporadic cases, blood eosinophilia is a common feature; this is a variable finding in endemic cases. The curious geographical distribution of EMF in the tropics remains unexplained.

Africa↗

Phonomechanocardiographic findings in endomyocardial fibrosis.

Endomyocardial fibrosis (EMF) is a severe restrictive cardiomyopathy characterized by the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of one or both ventricles. The disease results in endocardial rigidity, atrioventricular valve dysfunction and progressive obliteration of the ventricular cavity. The clinical and pathological findings of EMF have been well established and definitive diagnosis can be made by selective cineangiocardiography. However, there are few reports concerning the phonomechanocardiographic (PMCG) patterns in this condition. In this paper we describe the PMCG findings in 12 patients with EMF which is now recognized with increasing frequency in the Northeast of Brazil.

Adult↗

[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↗

Histomorphologic characteristics of endomyocardial fibrosis: an endomyocardial biopsy study.

Endomyocardial biopsies from right, left, or both ventricles were performed in 13 angiographically documented cases of endomyocardial fibrosis. The endocardium was appreciably thickened due to acellular hyalinized collagen tissue in all cases. Variable amounts of elastic tissue intimately admixed with fibrous tissue were recognized. A "zonal layering" pattern of the endocardium was absent. Thrombus, inflammatory cells, and granulation tissue at the endomyocardial interphase, and eosinophils within the biopsy were not seen. In addition, lymphomononuclear interstitial inflammatory infiltrates were seen in five cases.

Adolescent↗

[Diagnosis of endomyocardial fibrosis with endomyocardial biopsy].

Ten cases of endomyocardial fibrosis (EMF) were diagnosed with angiocardiography in showing a huge right atrium' tricuspid regurgitation and obliteration of right ventricular apex. The diagnosis was confirmed in 9 of them pathologically after right ventricular endomyocardial biopsy (EMB) in showing striking thickening of endocardium with presence of fibrosis. The remaining case was not confirmed with EMB. It is concluded that EMB is a quite useful method in the diagnosis of EMF. The cause of misdiagnosis in the tenth case was discussed.

Adolescent↗

[Diagnostic significance of phono-mechanographic medication of constrictive endocardial fibrosis (or endomyocardial fibrosis)].

The phonomecanographic investigation of 12 patients with FEC allow us to recognize diagnostics signs of great interest. 1. The ventricular aspect of the jugular veinous pulse involving a positive systolic wave and a diastolic aspect in "dip-plateau" is a chief indication of a right ventricular fibrosis. This indication is more significant of FEC when the nadir of the dip is far above the base line, when the dip-rising time is short, when the telediastolic ratio is high (more important than 0.40) when the electro-mechanical latency time is prolonged and when the isovolumetric relaxation time is normal. 2. The apexogram aspect in diastolic dip-plateau is a capital indication for a fibrosis of the left ventricule. This indication is more significant when the nadir of the dip is above the baseline, when the telediastolic ratio is high (more important than 0.30) when the electromechanical latency time and the isovolumetric relaxation time are normal. 3. The protodiastolic endocardial vibrancy recorded during the dip climbing and showing the features we described, seems to us to be a very important sign. This vibrancy can be preceded or/and followed by a short and early diastolic murmur of low frequency, always finishing at the end of the dip. 4. Recording of pulmonary valves or atrio-ventricular murmur is an important diagnostic feature in an adiastolic syndrome. 5. Recording of a pulmoanry infundibular impulse is an indication for a right ventricular fibrosis. This indication is very significant if the tracing is a dip-plateau.

Adolescent↗

Surgical palliation for endomyocardial fibrosis: early results.

Endomyocardial fibrosis is a fatal disease of obscure aetiology which is notably prevalent in Uganda, Brazil, Kerala State in India, and certain other parts of the world. The main disability in the disease results from fibrotic obliteration of ventricular chambers and insufficiency of the mitral and tricuspid valves. The poor response to medical treatment and the bleak prognosis of the patients led to the introduction of endocardiectomy and replacement of the atrioventricular valves as a palliative procedure by Dubost and colleagues. The present report on the early results in 17 patients from Kerala State would support the continued use of this palliative procedure in endomyocardial fibrosis with severe disability.

Adolescent↗

Surgical treatment of endomyocardial fibrosis.

Thirty patients with endomyocardial fibrosis were submitted to endocardial decortication and atrioventricular valve replacement between December, 1977, and October, 1981. There were 26 female and four male patients, ranging in age from 14 to 48 years (mean 32). Thirteen patients had biventricular disease, 14 had the right-sided form, and three had endomyocardial fibrosis confined to the left ventricle. All were in Functional Class III or IV (New York Heart Association classification). The hospital mortality was 20% (six cases). Among the survivors (mean follow-up 13 months), 23 of 24 were improved clinically. Postoperative hemodynamic and angiographic studies were performed in 15 patients. Two (6.6%) have definitive atrioventricular heart block. There were five (16.6%) late deaths. Operation for endomyocardial fibrosis should be considered a palliative procedure. Possible limitations include the need for a valve prosthesis, cardiac conduction disturbances secondary to endocardiectomy of the right ventricle, and the possibility of recurrence of the endocardial fibrosis. However, at present, operation seems to be the treatment of choice for this condition because (1) endomyocardial fibrosis is characterized by a grave prognosis and medical therapy is ineffective; (2) endomyocardial fibrosis is a disease in which only the heart is affected, lesions in other organs being the result of passive congestion; (3) systolic performance of the heart is usually only slightly depressed; and (4) the surgical procedure is easily performed, so that the mortality is acceptable.

Adolescent↗

Surgical treatment of endomyocardial fibrosis: a new approach.

Endomyocardial fibrosis has been treated surgically for many years. For complete removal of fibrosis from both ventricles by the classic technique, each atrioventricular (AV) valve was removed and replaced with a prosthesis. Relapse of endomyocardial fibrosis has not been observed after surgical correction. Reoperations have been carried out because of complications of valve prostheses. A new surgical technique for removal of ventricular fibrous tissue with preservation of the mitral and tricuspid valves was used in nine consecutive patients with endomyocardial fibrosis. Initial results show a reduction of pulmonary hypertension, mean right and left atrial pressures and end-diastolic pressures in both ventricles. Tricuspid annuloplasty was performed in seven patients and mitral annuloplasty in five. No valve prosthesis was used. There was no death and New York Heart Association functional class improved from class III or IV in the preoperative period to class I or II in the postoperative period. These data suggest that resection of endocardial fibrous tissue can be indicated early in the clinical course and performed with preservation of the AV valves.

Adult↗

Endomyocardial fibrosis in Africa.

Endomyocardial fibrosis (EMF) is a disease of the rain-forest belt in Africa. There is general agreement as to its pathology in the acute phase, but this is difficult to diagnose clinically. The aetiology is still unknown although there are reports which suggest that eosinophilic endomyocardial disease may be the cause. Further studies are needed to define EMF in its acute stage and find out how chronic EMF evolves. A longitudinal study on young people with eosinophilia and a comparative study of two villages, one in an endemic zone and the other in a zone where EMF is uncommon, will also be helpful in identifying its cause. The most promising form of treatment at present is surgical.

Africa↗

[Doppler echocardiography in endomyocardial fibrosis].

Twelve patients with endomyocardial fibrosis with angiographic and/or histologic corroboration were studied with Doppler echocardiography with the purpose of describing the echocardiographic features and identify the affected sites. The average age was 41 years (range 16 to 59 years), 2 men and 10 women. Three patients (25%) had isolated right ventricular involvement, one patient (8%) left ventricular, 8 patients (66%) both ventricular. Our Doppler echocardiographic findings were: right atrium enlargement (91%), right ventricle outflow dilatation (83%), paradoxical septal motion (83%), left atrial enlargement (33%), mitral and tricuspid valve prolapse (50%), pericardial effusion (41%), mitral regurgitation (75%), tricuspid regurgitation (100%), apex obliteration (50%) and a restrictive type flow pattern (50%). Doppler echocardiography is a useful method for the diagnosis of endomyocardial fibrosis, the finding of normal or small ventricles associated with apex obliteration and enlarged atria, mitral or tricuspid regurgitation and a restrictive type flow pattern are characteristics of this disease. In our population, the isolated or predominantely right ventricular involvement is the most common finding as it represented 83% of the cases.

Adolescent↗