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At least 19 recordsLinked to original sources
PubMed1976

Prognostic value of the electrocardiogram in endocardial fibroelastosis.

Nine children with endocardial fibroelastosis were followed from the time of admission with congestive heart failure until either death or discharge. Review of multiple clinical features showed that only the electrocardiographic pattern could be correlated with death or survival. The presence of a delayed transition zone with anterior force loss on the initial electrocardiogram ('infarct pattern') was noted in all the children who died. Progression of these changes with a pattern of anterolateral 'infarct' in two and inferior wall 'infarct' in two occurred before death. Necropsy on three of the four children confirmed the diagnosis of endocardial fibroelastosis. There was extensive fibrosis and thinning of the left ventricular myocardium as well as involvement of the mitral valve structures. Review of published cases supports the view that an 'infarct' pattern in a child with endocardial fibroelastosis is usually associated with death and that this pattern is a negative prognostic sign for survival.

Cardiomegaly↗
PubMed1977

Ultrasonic studies on endocardial fibroelastosis.

Four patients with endocardial fibroelastosis were studied by two-dimensional and M-mode echocardiography. The two-dimensional echocardiography showed the characteristic echo from the abnormally thickened endocardium of the left ventricle in every case. Of the 4 cases, 2 were still suffering from congestive heart failure at the time of the study and the other 2 had been already free from clinical symptoms. In the former group, the ultrasonic findings revealed an enlargement of the left ventricle (large left ventricular dimension index) and poor movement of the anterior mitral leaflet which was indicated by a decrease in the diastolic descent rate. The latter group presented almost normal findings concerning the above points. The ultrasonic method is thus proved to be very useful for the diagnosis and evaluation of clinical conditions in cases of endocardial fibroelastosis.

Child↗
PubMed1975

Coronary and endocardial fibroelastosis of the ventricles in the hypoplastic left and right heart syndromes.

In an autopsy material of 29 cases of the hypoplastic left heart syndrome coronary fibroelastosis was found in 1 case, endocardial fibroelastosis in 8 cases. Figures for 10 cases of the hypoplastic right heart syndrome were 6 cases of coronary fibroelastosis and 1 case of endocardial fibroelastosis. Age ranged from stillborn up to 11-1/2 months. Coronary and endocardial fibroelastosis seemed to be mutually exclusive localizations of congenital fibroelastosis since in our material they did not occur together in the same hearts. In hypoplastic right hearts coronary fibroelastosis was either restricted to the right coronary artery (right circumflex and posterior interventricular branch), or it was found also in the left coronary artery (anterior interventricular branch), with the most serve affections always being situated in the right one. In the only case of coronary fibroelastosis among the hypoplastic left hearts the condition was limited to the anterior interventricular branch of the left coronary artery which communicated with the hypoplastic left ventricle by a fistula. Coronary fibroelastosis was exclusively found in branches supplying the hypoplastic right ventricle and/or in a branch connected by a fistula to the hypoplastic left or right ventricle. Endocardial fibroelastosis was generally found in hypoplastic left ventricles with either no outflow or with severe outflow obstruction. A theory concerning the aetiology of both coronary and endocardial fibroelastosis of the hypoplastic ventricles is proposed. It is argued that development of fibroelastosis may in both localizations be caused or favoured by the coincidence of two factors: abnormal haemodynamic conditions and poor oxygenation of blood and tissues. Observations made in a reference material of 35 hypoplastic left and 24 hypoplastic right hearts were in accordance with this view.

Arteries↗
PubMed1975

Heredity in primary endocardial fibroelastosis.

Twenty-six cases of endocardial fibroelastosis were collected from three hospitals in Manchester over a ten-year period. Nine cases occurred in 4 families and these are discussed in detail. X-linked recessive inheritance seems likely in one family in which two probable female carriers had subarachnoid haemorrhages. In a second family an apparently normal man produced two children with endocardial fibroelastosis by different mothers suggesting autosomal dominant inheritance with incomplete penetrance. Autosomal recessive inheritance may be involved in the remaining two families but this was not associated with consanguinity. Genetic heterogeneity is evident in endocardial fibroelastosis and the majority of cases occur sporadically. An accurate family history is therefore necessary but it is difficult to give precise recurrence risks in sporadic cases.

Cardiomegaly↗
PubMed1977

Endocardial fibroelastosis and Niemann-Pick disease.

The concurrence of endocardial fibroelastosis and Niemann-Pick disease is described. This appears to be the first described case of endocardial fibroelastosis in association with a lipid storage disorder.

Endocardial Fibroelastosis↗
PubMed1978

Familial nonobstructive cardiomyopathy with endocardial fibroelastosis beyond infancy.

A 10-year-old boy with congestive heart failure died in five months in spite of comprehensive medical treatment. Autopsy showed patchy areas of endocardial fibroelastosis of the left ventricle. The sister of this patient had followed a similar course at 13 years of age with death within six months of the onset of congestive failure. Her postmortem examination also showed endocardial fibroelastosis. The clinical presentation of familial endocardial fibroelastosis in the preteen and teenage years is a rare event. Probably the endocardial fibroelastosis was secondary to a familial nonobstructive cardiomyopathy.

Adolescent↗
PubMed1976

[Endocardial fibroelastosis (E.F.) and its differential diagnosis].

The endocardial fibroelastosis (EFE) is the most frequent cardiomyopathy. This disease is characterised by endocardial hyperplasia due to proliferation of elastic and collagenous fibres. There are primary and secondary forms. Within the primary form, the infantile form is the most frequent and of greatest importance to the pediatrician. This form is more a syndrom than a distinct disease. It is a reaction of the endocard due to several noxes. Lately a possible viral etiology is being discussed e.g. Parotitis, Coxsackie or other viruses. Clinical criteria for diagnosis are: cardiomegaly, left ventricular hypertrophy seen in 97% in the ECG, the absence of a murmur (or a soft apical mumur) absence of cyanosis and absence of systemic disease. Differential diagnosis is mainly between fibroplastic parietal endocarditis (FPE), cardiovascular collagenosis (CC) and endomyocard fibrosis (EMF). In FPE thrombosis is frequent and typically there is eosinophilia. CC is found in South Africa and is characterised by edema and fibrinoid necrosis. MEF is present mainly in Uganda, Nigeria and South India, characterised by endocardial fibrosis, valve involvement and eosinophilia. The obstructive hypertrophic cardiomyopathy is characterised by a pronounced cardiomegaly, insufficient weight gain as well as dyspnea and cyanosis. Catheterization shows a gradient across one or both of the outflow tracts due to hypertrophic subaortic or subpulmonic stenosis. Therapy of EFE consists in treating the cardiac decompensation and according to the severity of the disease, in steroids.

Cardiomyopathies↗
PubMed1979

[Echocardiographic findings and function analysis of the left ventricle in infants with endocardial fibroelastosis (author's transl)].

7 infants with the clinical picture and the typical hemodynamic and angiocardiographic findings of endocardial fibroelastosis were studied echocardiographically. The echocardiograms were digitized and analyzed by the method of Gibson and Brown and compared with those of 8 normal infants. The echoes of EFE-patients showed a nearly twice as large LV-diameter. The mitral valve was displaced posteriorly, the septal motion in 4 of them abnormal. The shortening fraction was significantly reduced, the LPEP/LVET quotient increased. The comparison of LPEP with the Q to mitral valve closing interval revealed a considerable prolongation of isovolumetric contraction time. The result of computer analysis was a decrease of peak Vcf and maximum lengthening rate. The echocardiographic pattern is typical but not pathognomonic, it gives no prognostic information.

Echocardiography↗
PubMed1977

[Endocardial fibroelastosis. Pathologic and clinical findings in 20 cases (author's transl)].

Pathologic and clinical findings in 20 infants with Endocardial Fibroelastosis are reported. Ten cases were considered as secondary Fibroelastosis due to left heart structural anomalies. Purpose was to find some clinical criteria to establish differential diagnosis between primary and secondary forms. QRS voltages in electrocardiogram were higher in primary forms and QRS axis in the frontal plane had more rightward deviation in secondary ones.

Diagnosis, Differential↗
PubMed1977

Histologic and ultrastructural features of primary and secondary endocardial fibroelastosis.

The average size of elastic fibers in thickened left ventricular endocardium was much larger in four patients with congenital endocardial fibroelastosis (EFE) than in six patients with acquired EFE (secondary to ischemic heart disease in two patients, to prosthetic cardiac valves in three, and to irradiation of the chest in one). Both components of normal elastic tissue (central, amorphous cores, and peripheral microfibrils) were present in endocardial elastic fibers of each patient. Ultrastructural identification of elastic fibers was greatly facilitated by staining with silver tetraphenylporphin sulfonate.

Adult↗
PubMedDate not supplied

[Radiological and angiocardiographical aspects of primary endocardial fibroelastosis in children (author's transl)].

In this article we try to show the value of radiography and angiocardiography in primary endocardial fibroelastosis. The 20 CASEs studied in this report, were angiocardiographically and/or necropsically proved. In spite of different opinions, we think that it is possible to reach an approximate diagnosis of this condition if we use the clinical and angiohemodinamic examinations. On the other hand, it is necessary to point out that pathology is characteristic. The most important radiological signs were cardiomegaly, together with venocapilar congestion, both of variable magnitude, and dependent on the degree of cardiac insufficiency. By means of angiocardiography we could appreciate a great dilatation and hypertrophy of the left ventricle and a reduction of the mobility and distensibility of this cavity along the cardiac cycle.

Angiocardiography↗
PubMed1978

Endocardial fibroelastosis: myocardial and vascular alterations associated with viral-like nuclear particles.

Although clinical, immunologic, and experimental evidence exists implicating in utero viral infection of the myocardium in the development of primary endocardial fibroelastosis, the infectious etiology of this condition remains somewhat controversial. To date, specific features of viral myocarditis and morphological demonstration of viral particles have not been described in EFE. The present case is the first in which extensive light microscopic and ultrastructural analysis of the myocardium revealed abnormalities consistent with a primary viral myocarditis associated with typical EFE. These alterations consisted of chronic myocardial inflammation, extensive interstitial fibrosis, severe degenerative changes in myocardial cells, and a marked proliferation of endothelial cells in large nad small intramyocardial vessels leading to vascular occlusions. In support of the infectious etiology of this disease, similar features were noted in skeletal muscle. Most interestingly, viral-like particles were observed in many myocardial and endothelial nuclei. Although we are not absolutely certain of the viral nature of these particles, their appearance suggests viral assoicated material. We propose that the presence of these particles in this case in association with the other morphological alterations in support for the viral etiology of EFE. The prominent vascular occlusion observed in the myocardium may be an important clue to the pathogenesis of fibroelastosis as either a primary or secondary disease.

Cell Nucleus↗
PubMed1979

Endocardial fibroelastosis found on transvascular endomyocardial biospsy in children.

Left ventricular endocardium obtained by transvascular endomyocardial biopsy from nine infants and children with various congestive cardiomyopathies showed thickening in five, with hyperplasia of endocardial cells and increase in elastic and collagen fibers (endocardial fibroelastosis). Based on the morphologic findings, we suggest a chronologic sequence of hyperplasia of smooth muscle (SM) cells followed by transformation and translocation. The sequence appears to be (1) proliferation of dark SM cells with many surface vesicles, many myofilaments, and fusiform densities; (2) possible proliferation of light SM cells containing fewer surface vesicles and fewer myofilaments than the dark SM cells; (3) transformation of SM cells to leiomyoid cells that resemble both SM cells and fibroblasts; and (4) transformation to typical fibroblasts. The cells producing the increased elastin and collagen are believed to be the SM cells and the leiomyoid cells.

Child, Preschool↗
PubMed1977

Sclerema neonatorum associated with systemic fibrosis and endocardial fibroelastosis.

A female newborn infant whose illness was diagnosed as sclerema edematosum on the basis of diffuse edematous skin induration having rapidly occurred until her death on the third day of life, was pathologically investigated. Autopsy revealed notable interstitial fibrosis of the subcuteneous adipose tissue, variable increases of fibrous connective tissue in various visceral organs, particularly prominent in the alimentary tract, as well as endocardial fibroelastosis of the heart. Although the skin lesion is similar to that of sclerema neonatorum presented by HUGHES et al., systemic fibrosis in the visceral organs has not been reported in the literature with respect of this disease. Furthermore, this case presented striking histopathological differences from scleroderma neonatorum and infantile subcutaneous fat necrosis in many respects. It is conceivable that this case is a peculiar disorder at least pathologically different from the previously known cutaneous indurative disorders of newborn infants. Possible pathogenesis of this case was briefly discussed.

Adipose Tissue↗