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PubMed2004

Clinico-pathological evaluation of restrictive cardiomyopathy (endomyocardial fibrosis and idiopathic restrictive cardiomyopathy) in India.

BACKGROUND: Restrictive heart disease is characterized by impairment of ventricular filling during diastole with preserved systolic function. The clinical and histopathological profile on endomyocardial biopsy of a cohort of patients with restrictive cardiomyopathy (RCM) is presented. METHODOLOGY: The medical records of patients presenting with heart failure with systemic congestion, subsequently diagnosed as restrictive heart disease after evaluation including cardiac catheterisation, were studied retrospectively to determine the clinical spectrum of restrictive cardiomyopathy. The diagnosis of RCM was made, based on systemic congestion with dilated atria and near normal ventricular size and function. Only patients who had an endomyocardial biopsy were included in the study. Patients with chronic constrictive pericarditis and secondary restrictive heart disease mainly amyloidosis were excluded from the study. RESULTS: All 52 patients had heart failure with normal or near normal left ventricular size and function. Based on right and left ventricle angiography, patients were classified into two groups. Group I with findings suggestive of EMF (n=30) and Group II no evidence of EMF on angiography i.e. 'idiopathic RCM' (IRCM) (n=22). Baseline characteristics were similar in the two groups. Echocardiography revealed typical features of endomyocardial fibrosis in Group I patients, with apical obliteration of right and left ventricular apices. Group II patients had no apex obliteration (except in four patients, who were misclassified and in whom angiography did not show apex obliteration). The Group II patients had features of IRCM in the form of normal left and right ventricular size and function with restrictive features of doppler filling along with dilated left and right atria. Angiocardiography in EMF patients showed isolated RV involvement in only two patients. In the remaining 28 patients, the obliterative changes were biventricular with RV involvement more severe than LV involvement. Angiographic findings in Group II (IRCM) patients were unremarkable with preservation of normal trabecular pattern and absence of obliterative changes. Mild atrioventricular regurgitation was present in 10/22 patients. Histopathological examination revealed that endocardial thickening was more common (77% vs. 23%) in EMF patients. The presence of myocyte hypertrophy (70-80%), myocytolysis (40-50%) and interstitial fibrosis (46-56%) were similar in both groups. CONCLUSIONS: The majority of our patients had biventricular EMF. A significant number of patients had clinical hemodynamic features of restrictive heart disease but no evidence of EMF on angiography. These IRCM patients had similar clinical profiles to EMF but on endomyocardial biopsy the endocardial thickening was minimal and seen in few patients (5/22).

Adolescent↗
PubMed1991

[Evaluation of endomyocardial fibrosis by two-dimensional echocardiogram. Analysis of the severity and correlation with angiocardiography].

PURPOSE: To evaluate the capacity of two-dimensional echocardiogram (2D-Echo) to establish the severity of endomyocardial fibrosis (EMF) in comparison with angiography (Angio). METHODS: Twelve patients with EMF were prospectively studied by both: 2D-Echo and Angio. The mean age was 24.6 +/- 6.6 years (14 to 35), with 8 women and 4 men. To compare the severity by the 2 methods the obliterative process was graded according to its extension relative to the long axis of the respective, in diastole, as follows: 1 + only apical: 2 + less than 50% and 3 + greater than or equal to 50% from the apex to the atrioventricular valve level. RESULTS: All patients (100%) had biventricular disease by Angio, with right ventricle (RV) predominance in 5 (42%), left ventricle (LV) predominance in 1 (8%) and balanced in 6 (50%). By 2D-Echo the se numbers were: 11 (92%), 5 (42%), 1 (8%) and 5 (42%), respectively. The degree of concordance between 2D-Echo and Angio was 100% for the RV and 75% for the LV (p less than 0.005). In only 1 patient the LV apical involvement (+) was not identified by 2D-Echo. Mitral insufficiency (MI) was detected in 7 (58%) and tricuspid (TI) in 11 (92%) patients by Angio. These patients, expect one, had the corresponding atrial area greater than 18 cm2 by 2D-Echo. It was observed a statistically significant correlation between the atrial area and the degree of AV valve insufficiency (MI p less than 0.005; TI p less than 0.05). Paradoxical movement of the interventricular septum (IVS) was noted in 6 (50%) patients, associated with predominant RV disease, severe TI and mild or absent MI. CONCLUSION: The 2D-Echo, a valuable non-invasive method to diagnose and to grade severity of the fibrotic involvement of the ventricles by EMF, has a good correlation with Angio.

Adolescent↗
PubMed1996

[The restrictive syndrome in endomyocardial fibrosis and the effect of enalapril based on acute drug test data].

6 hours after the second dose (5 mg) of enalapril 9 males and 6 females (mean age 34.93 +/- 1.03 years) with endomyocardial fibrosis (EMF) underwent ECG and pulsed Doppler echocardiography to study enalapril effect on cardiohemodynamics. As shown by ventricular diastolic function and systolic flow in the pulmonary artery with estimation of mean pressure in the pulmonary artery according to Kitabatake, enalapril (renitek) affects positively cardiohemodynamics of EMF patients: improvement of ventricular diastolic function occurred in line with a significant fall in the pulmonary artery pressure. It is noted that application of Doppler echocardiography is essential for detection of restriction syndrome.

Adult↗
PubMed2001

Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis.

Significant eosinophilia and even eosinophilic tissue infiltration has been associated with a variety of clinical disorders including allergic and immunodeficiency states, drug reaction, infection, parasitic infestation and malignancy. Eosinophilia without an underlying aetiology and with multi-organ dysfunction has been designated idiopathic hypereosinophilic syndrome. We report a case of endomyocardial fibrosis with MRI findings.

Endomyocardial Fibrosis↗
PubMed1976

Eosinophilia and endomyocardial fibrosis.

A 12 year old boy died after a 3 year course of recurrent and progressive heart failure. His cardiac symptoms began with a marked leukocytosis (white blood cell count 188,500/mm3) due to eosinophilia (90 percent). In 6 months, the leukocytosis and eosinophilia subsided, but the patient's heart failure progressed over the next 2 1/2 years. At autopsy there was no evidence of leukemia, but a severe endocardial fibrosis extending into the myocardium was found. The origin of endomyocardial fibrosis, although unknown, appears to have been related to the eosinophilia in this patient.

Child↗
PubMed1977

Echocardiographic diagnosis and evaluation of cardiomyopathies: idiopathic hypertrophic subaortic stenosis, Chagas' heart disease and endomyocardial fibrosis.

Echocardiographic investigations on patients with hypertrophic cardiomyopathy with obstruction have been detailed and compared with the changes found in sixty patients with chronic Chagas' 'cardiomyopathy'. These changes are similar to those encountered in congestive cardiomyopathy. Endomyocardial fibrosis is rare in Venezuela, but six patients have been found in that country and the echocardiographic changes in one of these patients has been included in this study.

Cardiomyopathies↗
PubMed1983

Renal lesions in the obliterative cardiomyopathies: endomyocardial fibrosis and Loffler's endocarditis.

Electron and, or light microscopic examination of paraffin embedded renal tissue obtained from necropsies on eleven patients with obliterative cardiomyopathy showed a variety of abnormalities. Glomerular lesions present in patients with endomyocardial fibrosis included capillary wall thickening, basement membrane duplication, mesangial expansion and interposition, intraluminal fibrin and dense subendothelial deposits. These changes, some of which have been reported earlier in patients with Loffler's endocarditis, probably result from deposition and organisation of immune complexes.

Adolescent↗
PubMed1982

Electrocardiographic abnormalities in Ebstein's anomaly. Deductions based on comparison with endomyocardial fibrosis.

Probable causes of ECG abnormalities in Ebstein's anomaly were investigated by comparing tracings from 18 young patients with the disease (group I) and 20 age-matched patients with a morphologically similar cardiopathy: right ventricular endomyocardial fibrosis (group II). Tall p waves (greater than or equal to 2.5 mm) occurred in about a third of the patients in each group and were attributable to right atriomegaly. 8 patients, 4 from each group, had prolonged P-R intervals (greater than or equal to 17 s) resulting from increased P-R segment (7 cases) and prolonged P-wave duration (4 cases). Right bundle branch block was, however, more prevalent in group I (44%) than in group II (5%), and is thought to result mainly from a paucity of conduction fibres in the atrialized right ventricle in Ebstein's anomaly and partly, in group II especially, from septal fibrosis. In both groups R-wave deflections in V3R and V1 were reduced, probably because of a clockwise cardiac rotation and paucity of right ventricular muscle mass.

Adolescent↗
PubMed2003

Endomyocardial fibrosis in infancy.

The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age.

Endomyocardial Fibrosis↗
PubMed2004

Multiple microembolic borderzone brain infarctions and endomyocardial fibrosis in idiopathic hypereosinophilic syndrome and in Schistosoma mansoni infestation.

We report two cases of multiple distal borderzone ischaemic strokes associated with hypereosinophilia due to idiopathic hypereosinophilic syndrome in one patient and to acute Schistosoma mansoni infestation in the other. Endomyocardial fibrosis (EMF) was documented pathologically, in one case at autopsy and in the other after cardiac surgery; and by cardiac CT, with initially negative echocardiography, in one patient. These observations suggest that so called borderzone infarcts may be due to microembolisms and that, in the context of hypereosinophilia, EMF diagnosis warrants complete cardiac investigation including cardiac CT and repeat echocardiography.

Adult↗
PubMed1990

[Endomyocardial fibrosis. Course in patients undergoing clinical and surgical treatment].

PURPOSE: To follow-up a group of patients during a period of time after either clinical or surgical approach. PATIENTS AND METHODS: A hundred and twenty-one patients were studied retrospectively with endomyocardial fibrosis (EMF) for a period that varied from one month to 11 years (mean = 32 months). Upon entrance, patients had from 5 to 64 years of age (mean = 30), being 41 male and 80 female. Biventricular involvement were present in 70 cases, whereas 36 showed pure left and 15 pure right ventricular involvement. The patients were divided in two groups: 62 treated clinically, and 59 surgically, the latest defined as fibrosis resection added to atrioventricular valve rebuilding or replacement. All surgical cases had a prior III to IV functional class (NYHA). RESULTS: In the clinical group there were 24 fatalities, 21 who were in class IV, 1 in class III, and 2 in class II. In the surgical group there were 18 fatalities, 12 early and 6 late. CONCLUSIONS: (1) All the patients who survived the operation showed functional class improvement; (2) surgical mortality decreased in the latest years; (3) clinical treatment is indicated to patients in classes I and II, groups with low mortality rates; (4) surgical treatment is indicated to patients in classes III and IV, provided that clinical mortality is high in these groups of patients.

Adolescent↗
PubMed2002

[Angiotensin converting enzyme inhibitor enalapril in the treatment of endomyocardial fibrosis in patients with lymphogranulomatosis subjected to radio- and chemotherapy].

Echocardiography was used in the study of 40 patients with stage IIA-IVB lymphogranulomatosis. In 2-6 years before the study all patients had been treated with combination radio- (36-44 Gy) and chemotherapy. Echocardiography revealed endomyocardial fibrosis and signs of cardiac remodeling (reduced left ventricular dimensions and volumes, decreased myocardial mass and impaired diastolic function). The patients were divided into 2 groups. Patients of group 1 were given enalapril (5-10 mg/day), of group 2 - potassium and magnesium aspartate and inosine. In 2 months 68% of patients in group 1 demonstrated improvement of structural and functional state of the heart, no such changes occurred in group 2.

Adolescent↗
PubMed1989

[Mitral valve insufficiency caused by endomyocardial fibrosis of the left ventricle. A case report].

A 38-year old female patient, who had asymptomatic heart disease for seven years, developed progressive and fatal heart failure in eight months. The diagnosis of mitral valve insufficiency was made five months before death. The necropsy revealed that the etiology of mitral insufficiency was isolated left ventricle endomyocardial fibrosis with unusual involvement of the posterior cusp of the mitral valve.

Adult↗
PubMed1989

[Endomyocardial fibrosis].

Since the first case of endocardial resection I have performed in 1971, many similar cases have been operated on in the world, principally in Equatorian areas such as Africa, Northeastern Brazil and Southern India. In caucasian race, cases are less frequent but are similar to the other ones: in fact, endomyocardial fibrosis which was described by Davies in 1948 in Africa and Löffler's fibroplastie endocarditis, associated with hypereosinophylia (1936) are similar and respond to the same treatment. Our purpose in this paper is to present the long term results of the 34 cases operated at the Broussais Hospital in Paris since 1971. There were 21 males and 13 females, mean age 32 years (8 to 64), 22 were caucasians, 12 black africans. The sites of resection were 8 right ventricular, 4 left and 22 biventricular. Valve replacement was monovalvular in 11 cases and bivalvular in 23, mainly with bioprostheses. There were 7 early deaths and 6 late deaths, 2 after 30 months and 4 after 45, 60, 84, 85 months; only 2 of them were due to cardiac causes. We have not observed any case of recurrence. All surviving patients were in class I/II of N.Y.H.A. despite the fact that 4 have had valvular replacement due to alteration of the bioprosthesis.

Endomyocardial Fibrosis↗