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[Endomyocardial fibrosis (its clinico-echocardiographic characteristics)].

To compare clinical and echocardiographic features in patients with restrictive cardiomyopathy (RCMP), 15 patients (9 males and 6 females, mean age 34.93 +/- 1.03 years, duration of the disease 9 +/- 4.2 months) were examined using complete echocardiographic and doppler echocardiographic investigation in impulse regimen. Endomyocardial fibrosis was not obvious clinically, but should be suspected in dyspnea upon a weak exercise, undue fatiguability, tachycardia in normal arterial pressure and size of the heart. Echocardiographic indications, on the contrary, were rather specific. Endomyocardial fibrosis is characterized by diminished ventricular cavities, thickening of the endocardium and subvalvular structures, changed shape of ventricular cavity, echo-CG signs of passive pulmonary hypertension, diastolic dysfunction of the left and right ventricles.

Adult↗

Electron microscopic changes in tropical endomyocardial fibrosis.

Electron microscopic examination of cardiac tissue from seven patients with tropical endomyocardial fibrosis showed the endocardium to be replaced by maturing granulation tissue containing haphazardly arranged collagen bundles, smooth muscle cells, blood vessels and degranulated mast cells. Mature collagen was present on the luminal aspect of the granulation tissue. Myofibres showed mitochondrial swelling, lamella bodies, focal myocytoplasmic clearing and collapsed intercalated discs. Small calibre blood vessels exhibited endothelial swelling, areas of basement membrane duplication and collections of neutrophilic and eosinophilic leucocytes. Eosinophils showed alteration in granule morphology. These changes are similar to the lesions reported in endomyocardial disease with eosinophilia.

Adolescent↗

[Surgery of endomyocardial fibrosis. Apropos of 32 cases].

From 1971 to 1984, 32 patients with endomyocardial fibrosis (EMF) were treated by endocardial resection (decortication) and valve replacement. The population consisted in 20 men and 12 women (age ranged from 8 to 64); 19 patients were european and 13 african. All were symptomatic, 78 p. 100 in stages III or IV of the NYHA. Hypereosinophilia was detected in 21 patients and its cause was determined in 11 cases. Cardiac involvement was biventricular in 22 patients and monoventricular in 10 patients. Six patients died in the immediate postoperative period and 6 late deaths were observed, owing to extracardiac causes in 4. There were no recurrence of EMF. Despite a high mortality rate, the authors suggest that all symptomatic EMF should benefit from endocardial resection.

Adolescent↗

Pattern of cardiac calcification in tropical endomyocardial fibrosis.

Over a two-year period we investigated 55 patients with endomyocardial fibrosis, using two-dimensional echocardiography, color-coded echocardiography, and image intensifier fluoroscopy, to delineate the regional distribution of cardiac calcification. Thirty five patients (64%) were found to have calcification of the ventricular walls. Calcific deposits were seen in the right ventricle in 26(59%) patients and in the left ventricle in 16(44%) patients. All patients who showed calcification were in severe cardiac failure indicating late stage of the disease. When image intensifier fluoroscopy was taken as the gold standard, echocardiography had a sensitivity of 100% and a specificity of 98% in detecting cardiac calcification. The high incidence of calcification in the ventricular walls in the present study is apparently due to the systematic use of two-dimensional echocardiography and image intensifier fluoroscopy.

Adolescent↗

Angina-like chest pain and syncope as the clinical presentation of left ventricular endomyocardial fibrosis: a case report.

A 47-year-old woman complained of angina-like chest pain, near-syncope, and syncopal episodes of 17 years' duration. Physical examination was unremarkable. A 12-lead resting ECG showed symmetrically inverted T waves in the inferior and anterolateral leads. A graded treadmill exercise stress test precipitated angina-like chest pain accompanied by a near-syncopal episode associated with a systemic arterial pressure of 60/40 mm Hg. Echocardiography disclosed left ventricular apical obliteration. Left ventriculogram showed a typical "ace of heart'' shadow as well as filling defects and apical obliteration. Endomyocardial biopsy of the left ventricle diagnosed left ventricular endomyocardial fibrosis. Thus, angina-like chest pain and near-syncopal episodes should be added to the list of clinical manifestations of pure left ventricular endomyocardial fibrosis.

Chest Pain↗

Endomyocardial fibrosis: early and late results of surgery in 20 patients.

Twenty patients with endomyocardial fibrosis (EMF), the largest series reported to date, were operated upon between June, 1978, and June, 1980. Eleven were male and nine female, ranging in age from 6 to 23 years (mean 13.3 years). There were seven cases of right ventricular (RVEMF), six of left ventricular (LVEMF), and seven cases of bilateral EMF. All patients underwent endocardiectomy and atrioventricular valve replacement with a xenograft. Four patients had an additional valvular annuloplasty. There were four postoperative deaths (all bilateral EMF): two from low cardiac output and one each from hepatic failure and cerebral malaria. There was one late death from serum hepatitis. The other patients had a relatively difficult postoperative course, but none of the 20 patients atrioventricular block. The longest follow-up of the 15 survivors is 28 months (mean 16.7 months). All patients are symptom free. Three take digitalis and/or diuretics. Ten have been recatheterized from 6 months to 1 year after operation. Intracardiac pressures, the ventricular cineangiogram, liver, and heart size returned to normal in patients with LVEMF; in RVEMF, despite clinical improvement, most of these parameters remained abnormal. Of special interest were (1) our recognitions of an early type of LVEMF and (2) our surgical preservation of a thin juxta-annular rim of fibrosis in the right ventricle to avoid atrioventricular block. Operation is indicated in all patients with LVEMF, despite greater risk. Early intervention is advised in RVEMF to avoid irreversible liver damage and cardiac enlargement.

Adolescent↗

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↗

[Endomyocardial fibrosis. Presentation of a clinical case].

The authors report a case of right ventricle endomyocardial fibrosis in a 6-year-old boy. Particular attention is paid to the discussion of the new methods for diagnosing and treating these conditions, namely echocardiography, endomyocardial biopsy and surgery.

Child↗

Left ventricular endomyocardial fibrosis in India.

Clinical, radiological, electrocardiographic, haemodynamic, and cineangiographic features of left ventricular endomyocardial fibrosis are described in 8 patients seen during the 10-year period 1965-1975. Exertional dyspnoea was the commonest presenting symptom. Mild to moderate cardiomegaly was often present. The apex beat was never forcible. A loud third heart sound over the apex and an apical early systolic murmur with late systolic decresendo were characteristic of this desease. Chest X-rays films showed left atrial enlargement and pulmonary venous congestion. A pronounced rise in left ventricular end-diastolic pressure, severe pulmonary hypertension, and low cardiac index were seen in 6. Ventriculography showed an irregular, poorly contracting left ventricle with areas of dyskinesis and large end-systolic volume. Mitral regurgitation, when present, was not severe.

Adolescent↗

[Radiological findings in endomyocardial fibrosis].

PURPOSE: To determine through conventional radiology the type of ventricular involvement in endomyocardial fibrosis (EMF). METHODS: We analyzed 56 cases with EMF confirmed by angiocardiography and 9 by postmortem study, aged between 16 and 56 years (mean 32); there were 42 females. Only one radiologist analyzed X-rays without any knowledge of the cineangiography findings. RESULTS: The right side of the heart was primarily involved in 9 patients and the cardiac silhouette was characteristically globular and had oligemic pulmonary fields (66.66%). The cardiothoracic ratio was 0.62 +/- 0.11. Out of 9 patients, 8 were female. The left side of the heart was established as being primarily involved in 11 cases and simulated rheumatic mitral disease. The cardiothoracic ratio was 0.51 +/- 0.09 and there were increased pulmonary fields in 63.6%. The biventricular disease occurred in 36 cases. There were radiologic findings of right and left side. The cardiothoracic ratio was 0.63 +/- 0.06 and there was oligemic pulmonary fields in 38.8%, increased pulmonary fields in 33.3% and was normal in 27.7%. There were 4:1 females. CONCLUSION: The radiological study is fundamental in the initial diagnosis of EMF. The type of involvement could be done in 66.07% of all cases by chest X-ray.

Adolescent↗

[Quantitative assessment of left ventricular regional wall motion in endomyocardial fibrosis].

OBJECTIVE: To analyze left ventricular (LV) regional wall motion in patients with endomyocardial fibrosis (EMF). METHODS: The study comprised 88 patients, 59 of the female sex, with a mean age of 39+/-13 years (range, 9 to 65) and with echocardiographic and angiographic evidence of left ventricular EMF. The intensity of fibrous tissue buildup on contrast cineventriculography was classified as mild, moderate, or severe. The overall left ventricular ejection fraction (LVEF) was determined by using the area-length method on ventriculography. The motion was measured in 100 equidistant chords perpendicular to the centerline drawn in the middle of the final diastolic and systolic contours and normalized to cardiac size. Five left ventricular segments were analyzed: A--apical; AL--anterolateral; AB--anterobasal; IA--inferoapical; IB--inferobasal. Abnormality was expressed in units of standard deviation of the mean motion in a normal population of reference, comprised of 103 patients with normal LV according to clinical and electrocardiographic data, and angiographic standards. RESULTS: Mean LVEF was 0.47+/-0.12. Fibrous tissue buildup in the left ventricle was mild in 12 patients, moderate in 40, and severe in 36. The regions with the poorest ventricular wall motion were A (-1.4+/-1.6 standard deviation/chords) and IA (-1.6+/-1.8 standard deviation/chords) compared with that in AB (-0.3+/-1.9 standard deviation/chords), AL (-0.5+/-1.8 standard deviation/chords) and IB (-0.9+/-1.3 standard deviation/chords). No relation was observed between the intensity of fibrous tissue buildup and regional ventricular wall motion. CONCLUSION: A change in LV regional wall motion exists in EMF, and it is independent of the intensity of fibrous tissue buildup qualitatively assessed. Nonuniform involvement of the LV should be considered when planning surgery for this disease.

Adolescent↗

Relation of severe eosinophilia and microfilariasis to chronic African endomyocardial fibrosis.

Over a two-year period, 44 patients had an eosinophil count above the 97th centile. Thirteen of these 44 had heart disease presenting within six months of the onset of symptoms. Microfilariasis was the most likely cause of the raised total eosinophil in these 13 patients. In all, the raised eosinophil count we returned to normal by the use of diethylcarbamazine (Banocide). Eleven of the 13 were followed up and eight of them (73%) developed clinical features of cardiac constriction and tricuspid regurgitation. The mean duration of follow-up was two years. Limited cardiac catheterisation studies in six of the patients showed evidence of constriction or of tricuspid regurgitation. The clinical features of these eight patients were indistinguishable from those found in chronic endomyocardial fibrosis. This disease was further suggested by recurrent cerebral embolism in one, and a large pericardial effusion in another; and it was also present in the one patient to come to necropsy. Thus, microfilaria-induced eosinophilia, when high, is frequently associated with heart disease which appears to be chronic endomyocardial fibrosis many months after the eosinophilia has returned to normal.

Adolescent↗

Adenocarcinoma of the cecum as the first manifestation of ulcerative colitis complicated by primary sclerosing cholangitis and endomyocardial fibrosis.

A 47-year-old male Caucasian patient, with no previous relevant medical history, presented in September 1996 with persistent right lower quadrant abdominal pain. A tumor in the cecum was identified and the patient was submitted to ileocecal resection with ileocolic anastomosis. Histological examination showed a moderately differentiated adenocarcinoma. One year later he developed bloody diarrhea, urgency, and loss of weight. Based on clinical presentation and histology of large bowel biopsies, a diagnosis of ulcerative colitis (UC) was established. The previously resected surgical specimen was reevaluated, and lesions resembling UC were identified in the nonneoplastic mucosa. High levels of alkaline phosphatase and gamma-glutamyl transferase were detected. These alterations could be traced back to 1991. Endoscopic retrograde cholangiopancreatography was performed, showing diagnostic features of primary sclerosing cholangitis (PSC), and the patient was put on ursodeoxycholic acid therapy. In March 1999, he started to have progressive dyspnea and signs of cardiac failure. Endomyocardial biopsy was performed showing extensive lesions of endomyocardial fibrosis. This case illustrates a rather silent course of UC in the presence of PSC, and supports the postulated increased risk in the development of proximally located colorectal carcinoma in these patients. Additionally, the development of endomyocardial fibrosis constituted an unexpected finding, not previously reported in this setting.

Abdominal Pain↗

Premature opening of the pulmonary valve in endomyocardial fibrosis.

Two-dimensional and Doppler echocardiographic findings in a 67-year-old man with endomyocardial fibrosis (EMF) are described. The two-dimensional echocardiogram showed typical features of EMF, right ventricular endomyocardial calcification, a thickened right ventricular wall, obliteration of the apex of the right ventricle and marked dilatation of the right atrium. In addition, premature opening of the pulmonary valve was observed during late diastole. The Doppler echocardiogram revealed forward flow from the right ventricle to the pulmonary artery, indicating the conduit state of the right ventricle. These findings were supported by cardiac catheterization and autopsy. Thus, two-dimensional and Doppler echocardiography are useful not only in making the diagnosis, but also in understanding the hemodynamic condition in EMF.

Aged↗

Endomyocardial fibrosis with aortic, mitral, and tricuspid valve involvement.

We recently saw a patient with endomyocardial fibrosis of the left ventricle, with aortic, mitra, and tricuspid valve involvement. The patient resided in Madrid and had never been to Africa. There was no history of filariasis. The patient underwent left ventricle endocardiectomy, aortic and mitral valve replacement with tricuspid valve commissurotomy, and Carpentier annuloplasty. Prospective follow-up was uneventful. Pathogenesis and surgical features are discussed herein.

Adult↗

Mitral annuloplasty in endomyocardial fibrosis: an alternative to valve replacement.

The case of a patient with left ventricular endomyocardial fibrosis (EMF) causing severe mitral regurgitation is presented. Excision of the fibrotic tissue through the left atrium and mitral annuloplasty resulted in symptomatic relief and uncomplicated pregnancy. An X-linked congenital dermatological condition, Bloch-Sulzberger syndrome (incontinentia pigmenti), associated with chronic eosinophilia, was also present. This occurrence with EMF has not previously been reported. Atrioventricular valve reconstruction is a feasible alternative to valve replacement in EMF.

Adult↗