[Endomyocardial and myocardial fibrosis of unknown etiology].
Explore the source record for details and available documents.
Adult↗
SEARCH · PubMed Health
Results for “ENDOMYOCARDIAL FIBROSIS”
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Histopathology of resected myocardium and outcome of partial left ventriculectomy.
BACKGROUND: Since preoperative hemodynamics are not proven to be a predictor of effects of partial left ventriculectomy (PLV), myocardial histopathology may be better correlated with effects and outcome of PLV. METHODS: Myocyte size (micron) in the excised myocardium was measured in 338 patients undergoing PLV. Endocardial fibrosis, interstitial fibrosis, and inflammatory cell infiltration were enumerated as none = 0, mild = 1, moderate = 2, and severe = 3. These histopathologic observations were correlated with patients' postoperative survival. RESULTS: Reduced survival was seen in patients with advanced (> or = moderate) interstitial fibrosis in all patients (n = 338, p = 0.064) and in the subgroup with nonischemic etiology (n = 229, p = 0.0039). Although correlation between endocardial and interstitial fibrosis was significant (r = 0.55, p < 0.01), endocardial fibrosis failed to correlate with postoperative survival. While Chagas' disease was associated with severe inflammation and poor survival, the presence of inflammatory cell infiltration had no effect on survival in all patients combined (p = 0.943). Although most patients (n = 266, 79%) had myocyte diameter over 30 micron, those with less hypertrophy (< 30 micron, n = 70, 21%) had a tendency toward increased survival (p = 0.067) regardless of underlying etiology. CONCLUSION: Interstitial fibrosis may be an important factor in stratification of patients for repair (PLV) or replacement (transplantation). PLV may be more beneficial in patients with less hypertrophy, before develqpment of interstitial fibrosis. Endomyocardial biopsy might not predict the extent or variation in degree of interstitial fibrosis, which may be better evaluated by other metabolic or perfusion studies that measure overall myocardial histopathology and viability.
Adolescent↗
[Pathology of the papillary muscles].
Anatomoclinical findings in 21 cases of papillary muscle (PM) damage found in 4,000 consecutive autopsies are reviewed. Mitral PM muscles were involved in 17 cases, the posteromedial muscle in 11 patients, in 8 of these with necrosis (N) in 3 with fibrosis (F), in 2 with rupture. The mitral antero-lateral (MAL) was involved in 4 cases, by myocardial infarction (MI), fibrosis, hemorrhage and infectious endocarditis (IE). Both mitral PM were involved in two patients (MI and endomyocardial fibrosis). Tricuspid PM involvement was found in 4 cases. In two because of MI and the two remaining with fibrosis. In the 12 cases with MI microscopic examination showed, PM necrosis of variable magnitude: in the 7 cases with F, there was increased interstitial tissue by connective tissue; in the case with IE there was severe inflammatory reaction with microabscess formation and in one case, a small central longitudinal artery was damaged, a finding not previously described. Mitral PM infarction caused regurgitant murmurs, increase in heart size, heart failure and death from acute pulmonary edema or serious arrhythmias. There was no radiological evidence of left atrial enlargement. ECG findings were not useful in the diagnosis. Based on this anatomoclinical experience considerations are made about other diagnostic and surgical methods.
Humans↗
The restrictive cardiomyopathies.
In parallel with the rapidly developing interest in the diastolic properties of ventricular function in the 1970s, the restrictive cardiomyopathies have taken their place as the third major category of primary heart muscle disease. The restrictive cardiomyopathies are characterized by primary abnormalities of diastolic ventricular function with normal to near normal systolic performance and little or no increase in end-diastolic or end-systolic dimensions of either right or left ventricle. The restrictive abnormality of ventricular function can result from myocardial or endomyocardial disease, the etiologies of which may be known or unknown. Diastolic dysfunction that is essentially myocardial can be idiopathic (probably an enzymatic/metabolic disturbance), infiltrative (myocardial interstitium), or within myocardial cells (storage diseases). Diastolic dysfunction that results from endomyocardial disease is typified by endomyocardial fibrosis or the hypereosinophilic syndrome, although carcinoid, metastatic malignancies, radiation, and anthracycline toxicity may be accompanied by endomyocardial restriction. Echocardiography and Doppler ultrasound, MRI, and radionuclear techniques have been major advances in providing diagnostic precision, contributing materially to the clinical identification of the restrictive cardiomyopathies as well as discriminating certain of their etiologies.
Adult↗
The results of transvenous endomyocardial biopsy can frequently be used to diagnose myocardial diseases in patients with idiopathic heart failure. Endomyocardial biopsies in 100 consecutive patients revealed a substantial incidence of myocarditis.
Transvenous endomyocardial biopsy is an accepted method to evaluate cardiac transplant rejection, but the clinical diagnostic value of the technique for other forms of cardiac disease has not been established. We performed biopsies in 100 consecutive patients without significant complications. The pathologic diagnostic information obtained was judged to be useful to the clinician in 54 and not useful in 46 patients. In 74 patients with congestive heart failure of unknown etiology and a dilated heart, useful pathologic diagnoses included myocarditis, vasculitis, doxorubicin cardiomyopathy, and congestive cardiomyopathy. In most of the patients with biopsy findings of myocarditis there were no other clinical or laboratory findings indicating the presence of this disease, and the diagnosis of myocarditis would have been overlooked without a biopsy. In 26 patients in whom there was clinical evidence of constrictive or restrictive cardiovascular physiologic characteristics, useful biopsy diagnoses included radiation-induced cardiomyopathy, endomyocardial fibrosis, amyloidosis, or no myocardial disease; in the patients without myocardial disease thoracotomies were performed for constrictive pericarditis. Transvenous endomyocardial biopsy can provide clinically useful information in the evaluation of diseases of the myocardium.
Adult↗
[Anti-tachycardia surgery in ventricular arrhythmia].
UNLABELLED: Recurrent sustained ventricular tachycardia (VT) is associated predominantly with ischemic heart disease, mostly in the chronic phase after myocardial infarction. Potentially life-threatening and drug-refractory ventricular tachycardias are called malignant VT. In the Federal Republic of Germany, VT develops in about 3,000 to 5,000 patients per year from the 100,000 who survive a myocardial infarction. About 10% of these patients prove to be medically-refractory or additionally are considered candidates for aneurysmectomy or coronary revascularization. Overall, for the Federal Republic of Germany, there is a need for approximately 500 to 1000 antitachycardia operations each year. The morphologic substrate for malignant VT are ischemically-damaged inhomogeneously-structured arrhythmogenic areas. The morphology results in electrical inhomogeneity which predisposes to electrophysiologic reentry phenomena. NATURAL HISTORY: The survival rate of patients with malignant VT who are not surgically treated is 70% at one year and 20 to 40% at four years (Figure 1). In those in whom the tachycardia can be medically controlled, the prognosis is 10.5 times more favorable than in those with medically-refractory arrhythmias. In one study of 45 patients with recurrent, sustained VT, only 20% of those with medical refractoriness were free of renewed arrhythmic events after 30 weeks as compared to 90% whose treatment had been designated effective (p less than 0.0004) (Figure 2). According to a further study, for patients with drug-refractory VT, the probability for sudden death within four years was 55% as compared with 5% for those with medically-controlled VT (p less than 0.0002). SURGICAL TREATMENT: The concept of surgical treatment of malignant VT encompasses delineation of the arrhythmogenic area by means of endocardial mapping and surgical ablation. Arrhythmogenic areas are located mostly in the transition zone between the viable muscle and an aneurysm at the left ventricular endomyocardial septum. With mapping, by means of local measurements of activity times, impulse spread throughout the heart can be recorded in a cartographic system. The left ventricular endocardial activation should be determined during sinus rhythm and tachycardia and, with normothermic extracorporeal circulation the left ventricle is incised, mostly in the aneurysmatic antero-apical area, prior to sequential interrogation of the endocardial surface (Figure 3). As an alternative to point-for-point mapping, by means of multi-terminal electrodes, electrocardiograms can be obtained simultaneously from multiple positions. During tachycardia, the earliest activation can be found in the arrhythmogenic area (Figure 4); during sinus rhythm, in these areas, delayed, low-amplitude and fragmented signals are present (Figure 5). Macroscopically, endomyocardial fibrosis is a common finding. The arrhythmogenic morphologic substrate is either reduced or rendered a homogeneous scar without electrical activity. In this regard, techniques for endomyocardial resection have been described by Harken and Josephson. As an alternative procedure. Guiraudon introduced the encircling endomyocardial resection with which the pathologic reentry circuit can be blocked and the microvascular blood flow to arrhythmogenic areas eliminated. One modification, the partial encircling resection, appears to yield comparable effectiveness with less damage to left ventricular function (Figure 6).(ABSTRACT TRUNCATED AT 400 WORDS)
Electrocardiography↗
Endomyocardial biopsy in infants and children with cardiomyopathy.
We reviewed our experience of endomyocardial biopsy performed on 21 symptomatic infants and children with cardiomyopathy. Clinical congestive cardiomyopathy was noted in 18 patients, 2 had hypertrophic cardiomyopathy, and 1 a restrictive cardiomyopathy. The biopsy findings led to a diagnosis of hemochromatosis in one patient, Adriamycin cardiomyopathy in another, and lymphocytic myocarditis in a third. Five patients had features of endocardial fibroelastosis, one endomyocardial fibrosis, and a further one, a mitochrondrial abnormality. In 11 patients normal or nonspecific features were seen. There were 2 myocardial perforations, both patients being successfully resuscitated. Endomyocardial biopsy, although occasionally hazardous, may sometimes provide valuable information which may lead to a diagnosis, facilitate treatment, and be of prognostic value. Despite the low positive yield, it may still be indicated in selected patients, in view of the seriousness and often poor prognosis of this disorder: 5 of our study group subsequently died.
Adolescent↗
Endomyocardial biopsy--its evaluation in cardiac disorders.
Endomyocardial biopsies (EMBs) of 47 patients with various cardiac disorders were analysed. Clinically 26 patients were diagnosed as having dilated cardiomyopathy (DCM), 9 myocarditis, six restrictive heart disease and six as Duchenne muscular dystrophy. EMB showed normal histological picture in six cases of DCM and findings consistent with DCM in rest of the twenty cases. None of these biopsies showed evidence of myocarditis. Diagnosis of myocarditis could be confirmed on EMB only in three out of nine clinically diagnosed cases. Cases with restrictive heart disease showed normal biopsy in two, infarction in one, findings consistent with endomyocardial fibrosis in one and amyloidosis in two cases. EMB on six cases of Duchenne muscular dystrophy showed nonspecific histological changes. To conclude, in this series, cases of DCM did not show evidence of underlying myocarditis in endomyocardial biopsies.
Biopsy↗
[Endomyocardial biopsy: its value in tropical pathology. Apropos of 3 new cases].
The authors report their experience with three endomyocardial biopsies in cases of endomyocardial fibrosis; two of them were successful. The light and electron microscopical changes found were of two types: one showed the classical appearances of pure fibrosis, which was acellular and associated with degenerative changes in the heart muscle cell; the other type consisted of young fibrous tissue with numerous fibroblasts secreting a copious fibrillary substance which may have been the precursor of collagen fibres. Endomyocardial biopsy is, in experienced hand, a useful means of diagnosis in the field of tropical heart disease: it can be helpful in diagnosis, provided that the biopsy site is carefully controlled by anteriography and cathereisation; additionally, the opportunity to study the histological and electron microscopical changes which this technique affords should lead to a better understanding of the pathogenesis of those tropical cardiac disorders whose aetiology is yet to be established.
Adult↗
Endomyocardial biopsy.
Endomyocardial biopsy has been used more frequently over the past 10 years in an increasing number of centers in this country and abroad. When done by an experienced physician, it is as safe as routine cardiac catheterization. Although biopsy is not yet applicable in all cases of myocardial disease, many investigators have found this procedure valuable in specific circumstances, including cardiac allograft rejection, anthracycline-induced cardiomyopathy, and myocarditis. With this technique diagnoses can be made for various disorders including cardiac amyloidosis, sarcoidosis, hemochromatosis, and endomyocardial fibrosis. Although helpful in detecting an unsuspected condition or in formulating prognosis in some patients, biopsy is not diagnostically specific in patients with dilated or hypertrophic cardiomyopathy, because these diseases have no completely pathognomonic features under current examination methods. The proper practice of endomyocardial biopsy requires both technical proficiency and expert pathologic interpretation. As a research tool, biopsy will continue to yield new knowledge about myocardial disease and its treatment.
Biopsy↗
Reducing doxorubicin cardiotoxicity in the rat using deferred treatment with ADR-529.
The purpose of this study was to evaluate the optimal timing of ADR-529 administration to protect rats treated with doxorubicin (DXR) against drug-induced cardiotoxicity. Complete electrocardiographic monitoring (QRS complex, S alpha T segment and T wave) and the histopathological analysis of cardiac tissue were used to assess the degree of heart damage produced in female rats treated with ten i.v. doses of 1 mg/kg DXR over a period of 15 weeks; body-weight increase and survival were also analyzed to evaluate the toxicity of treatments. Cardiac alterations induced by DXR were compared with those occurring in animals receiving 20 mg/kg i.v. ADR-529 at 30 min prior to DXR administration, starting at the first, third, or sixth DXR dose and given until the end of the study (15th week). Rats treated with DXR were severely cardiomyopathic, showing progressive and irreversible ECG alterations (QRS-complex and S alpha T-segment widening and T-wave flattening) and marked degeneration of the myocardium (myocyte vacuolation, myofibrillar loss, and endomyocardial fibrosis). The most effective cardiac protection was provided by the administration of ADR-529 beginning with the first or third DXR dose. Delaying treatment with ADR-529 until the sixth DXR dose resulted in a significant reduction in its therapeutic action on heart damage. A significant difference in body-weight increase and survival was observed between the treatment groups: ADR-529 injected prior to the first DXR dose significantly protected animals from DXR toxicity, but this schedule was significantly more toxic than the administration of ADR-529 beginning with the third or sixth DXR dose. Taking into account the degree of cardiac protection and the toxicity of combination treatments, the results of the present study demonstrate the superiority of ADR-529 given prior to the third DXR dose over the other schedules tested. This finding suggests that significant protection against DXR-induced chronic cardiotoxicity in the rat can be obtained using deferred treatment with ADR-529.
Animals↗
Levels of cerium in the tissues of rats fed a magnesium-restricted and cerium-adulterated diet.
Cerium, a lanthanide, is the most abundant rare earth element present in monazite and is biologically active. It has been postulated that cerium toxicity in conjunction with magnesium deficiency causes tropical endomyocardial fibrosis, a restrictive human cardiomyopathy (Valiathan et al. 1989; Valiathan and Kartha 1990). Generally, lanthanides are known to be poorly absorbed (Durbin et al. 1956; Evans 1990). The present study was carried out to ascertain whether magnesium deficiency promotes accumulation of cerium in various organs of rats.
Animals↗
Fatal eosinophilic heart disease in a child with neurofibromatosis-1 complicated by acute lymphoblastic leukemia.
We present a pediatric case of neurofibromatosis-1 (NF-1) complicated by acute lymphoblastic leukemia and hypereosinophilia, which caused multiple end-organ damage. Although clinical symptoms such as fever and coughing were noted only 1 week before admission, the condition deteriorated rapidly with a fatal outcome prior to antileukemic therapy. A postmortem examination demonstrated extensive endomyocardial fibrosis with thrombotic occlusion and recanalization of the coronary arteries. Leukemic cell infiltration was not seen in the cardiac tissue. When eosinophilia is diagnosed in patients with NF-1, eosinophilic end-organ damage, particularly cardiac involvement, in addition to hematological malignancies, should be screened for in order to start medical treatment at the early stage of the disease.
Child, Preschool↗
Sequential magnetic resonance imaging findings in hypereosinophilia-induced encephalopathy.
Hypereosiophilia-induced encephalopathy (HE) is a rare but well-described clinical syndrome. However, serial magnetic resonance imaging (MRI) findings of HE have rarely been reported. We describe serial MRI findings of three patients with HE. The patients presented with acute confusion, focal neurological deficits and/or seizures. Eosinophils in repeated blood tests were more than 3000/mm3 in all the patients. Echocardiography in two patients showed findings consistent with eosinophilic endomyocardial fibrosis or global hypokinesia. The initial MRI revealed multiple high-signal lesions on T2-weighted images with gadolinium-DTPA enhancement on T1-weighted images, which were predominantly distributed in the border zone of the middle-anterior cerebral arteries and the middle-posterior cerebral arteries. The second MRIs taken prior to the initiation of steroid therapy showed that the lesions increased in size and number in the same area. The third MRIs performed long after the therapy showed that the lesions were shrunken. A brain biopsy specimen in one patient showed reactive gliosis following infarction with abundant intravascular eosinophils. The MRI-identified lesions in the patients with HE thus develop mainly in the border zone. The lesions occasionally increase in size and number and shrink if the eosinophilia is adequately treated. Although the nature of the MRI-identified lesions remains unclear, their pathogenesis may be related to multiple embolisms associated with concomitant cardiac abnormality and hypercoagulable state.
Adult↗
Pulmonary valve replacement with a mechanical prosthesis. Promising results of 28 procedures in patients with congenital heart disease.
OBJECTIVE: Pulmonary valve replacement is performed increasingly late after correction of Tetralogy of Fallot. Most reports deal with pulmonary allografts as the valvar substitute of choice, although late deterioration and reoperation(s) are the rule. Mechanical valves are scarcely reported and if so only because of complications. Although life-long anticoagulation therapy is indicated for mechanical prostheses, the chance of subsequent re-operations can be expected to be low. We report the results of 28 mechanical valve replacements in the pulmonary position. METHODS: A mechanical valve was implanted in 27 of 79 patients indicated for pulmonary valve replacement. Tetralogy of Fallot was the most common basic lesion. The results and follow-up were reviewed retrospectively, where death and re-operation were primary end points. Routine outpatient follow-up, including trans-thoracic echocardiography, was performed. RESULTS: Twenty-eight pulmonary valve implantations were done in 27 patients. Thirty-day hospital mortality was 1/28 (3.6%), because of a cerebro-vascular accident. One patient died late (2.8 years postoperatively). Median age was 33 years and the median interval between primary repair and insertion of the prosthesis was 26 years. Freedom from re-operation at 1 year was 100%. One valve had to be replaced 14 years after implantation because of malfunction due to ingrowth of endomyocardial fibrosis. No thrombo-embolic events were observed. CONCLUSIONS: Our series do not confirm the bad reputation of mechanical valvar prostheses in the pulmonary position. On the contrary they perform well and result in a much lower re-operation rate than can be expected and in fact is reported after allograft usage. No thrombo-embolic complications were noted. In our experience pulmonary mechanical valve prostheses do well.
Adolescent↗
[Cardiac pseudotumor revealing Behçet's disease].
INTRODUCTION: Cardiac thrombosis is a rare complication of Behçet's disease (BD), which may present as a cardiac tumor. Its discovery precedes, in half of the cases, the diagnosis of BD. The high mortality may be associated to postsurgical complications and/or an associated involvement of pulmonary arteries. CASE REPORT: We present the case of a 31 years old Caucasian French woman, with a history of venous thromboembolic disease, who had surgery after the discovery of a right ventricle tumor. That was an organised thrombus with endomyocardial fibrosis and a diagnosis of Behçet's disease was made after the surgery. The outcome was favourable under medical treatment associating corticosteroids, colchicine and antivitamin K (AVK), without relapse four years later. CONCLUSION: The discovery of an intracardiac mass in a young patient must evoke the diagnosis of cardiac thrombus and Behçet's disease, even in the absence of predisposing ethnic or geographic factor.
Adult↗
Right ventricular cardiomyoplasty: 10-year follow-up.
BACKGROUND: Chronically depressed right ventricular (RV) function presents an unsolved therapeutic challenge in cardiac surgery. Despite recent advances in medical and surgical therapies, prognosis remains poor and patient's quality of life and mortality are frequently unacceptable. The aim of this study is to present the first clinical report and long-term results of RV dynamic cardiomyoplasty applied in patients with RV failure caused by isolated RV cardiomyopathies. METHODS: Seven consecutive patients (5 males, 2 females; mean age, 40 +/- 9 years; range, 15 to 63 years) from a series of 113 cardiomyoplasty procedures performed at Broussais and Pompidou Hospitals were evaluated. The mean duration of follow-up was 10 +/- 3.5 years. All patients had predominant RV dysfunction, associated with tricuspid regurgitation in 6 patients. The cause of RV failure was arrhythmogenic cardiomyopathy (4 patients), ischemic (2 patients), and Uhl's disease (1 patient), and endomyocardial fibrosis (1 patient). Six patients were in preoperative New York Heart Association functional class III and 1 was in intermittent class III/IV. The mean preoperative ejection fraction (measured by isotopic technique) was 18% +/- 5.7% for the right ventricle and 40% +/- 13% for the left ventricle. Right ventricular dynamic cardiomyoplasty consists of wrapping the RV free walls with the left latissimus dorsi muscle flap. The distal part of the latissimus dorsi muscle is fixed to the diaphragm and then electrostimulated. Six patients required associated tricuspid valve surgery. RESULTS: There were no perioperative deaths. The mean duration of follow-up was 10 +/- 3.5 years. Six patients are alive with a remarkable quality of life, 4 are in New York Heart Association functional class I and 2 are in class II. One patient who was in New York Heart Association functional class II died in postoperative year 7 caused by stroke. At last follow-up, mean RV ejection fraction was 33% +/- 11.8% and left ventricular ejection fraction was 52% +/- 12.6%. CONCLUSIONS: The results of this long-term study demonstrate hemodynamic and functional improvements after RV cardiomyoplasty without perioperative mortality, no long-term malignant arrhythmias, and RV dysfunction related deaths. We believe that RV cardiomyoplasty, associated with tricuspid valve surgery when required, could be an effective treatment for severe RV failure.
Adolescent↗
Hypereosinophilic thrombus causing aortic stenosis and myocardial infarction.
Hypereosinophilia can cause severe endomyocardial fibrosis with subsequent restrictive cardiac disease and endocardial lesions. We present a case of a 37-year-old man with known hypereosinophilia, followed with yearly echocardiograms, who presented acutely with myocardial infarction and aortic outflow tract obstruction. At surgery, a broad based eosinophilic thrombus was found that had obstructed the aortic outflow tract. The possibility of the rapid emergence of such obstructive thrombotic lesions in hypereosinophilic syndromes warrants very close echocardiographic surveillance.
Adult↗