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At least 19 recordsLinked to original sources
PubMed1988

Fetal echocardiographic signs of congenital endocardial fibroelastosis.

Endocardial fibroelastosis is characterized by a porcelain-like thickening of the endocardium, resulting in a marked increase in echodensity of the endocardium, as well as ventricular dilatation and aortic atresia. With improvement in prenatal ultrasound, this condition can be suspected in utero on the basis of ventricular enlargement, poor ventricular contractility, and marked echodensity of the endocardial surface. We present two cases in which such conditions were found on prenatal M-mode echocardiography and two-dimensional directed pulsed Doppler. Ventriculomegaly and hypocontractility of the ventricle are, however, nonspecific for such conditions; the diagnosis can be made accurately only by pathology. When such findings appear on ultrasound, all efforts should be made to deliver the patient in a perinatal center for optimal neonatal surgery to improve the survival of the newborn.

Adult↗
PubMed2000

[Primary endocardial fibroelastosis].

Endocardial fibroelastosis is a disease characterized by a prominent proliferation of dense collagenous and elastic tissue in the endocardium with the occurrence of congestive heart failure and death in early life. There are, however, many recorded instances of infants surviving the initial illness and living up to or beyond puberty. The clinicopathological factors for good prognosis include the increase in myocardial mass of ventricle evaluated by ventriculography and the histopathologic evidence for compensatory myocardial cell hypertrophy in the biopsied myocardium.

Child↗
PubMed2003

Electron beam computed tomography appearance of endocardial fibroelastosis EBCT appearance of endocardial fibroelastosis.

Recent reports of endocardial fibroelastosis (EFE) have not reported the disease to be correctly diagnosed during the patients' life spans. Our purpose in this communication is to provide some feasible approaches toward correct diagnosis at the primitive stage and possible correlations to the prognosis. We analyzed five cases of EFE from 1997 to 2001. Four had pathology proven EFE. Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis. A case of anomalous left coronary artery originating from main pulmonary artery diagnosed EFE correctly before death by utilizing an EBCT. The second case was double outlet of the right ventricle with severe calcification and fibrosis shown on EBCT studies, while the third case had severe calcification over both apices. Both patients required heart transplantation. The fourth case, with a decreasing ejection fraction, was idiopathic hypertropic subaortic stenosis with mild calcification and fibrosis on the EBCT images. The last stationary case had severe aortic stenosis with trivial fibrotic change and calcification. We propose that EBCT may accurately help to diagnose EFE before pathology confirmation. The magnitude of calcification and fibrotic thickness in the myocardium of the EBCT imaging may predict the outcome of EFE.

Adult↗
PubMed2001

Doppler fetal echocardiography in endocardial fibroelastosis.

BACKGROUND: Endocardial fibroelastosis is a congenital heart disease known to cause congestive heart failure in early infancy. We report a case using new modalities for early prenatal diagnosis of endocardial fibroelastosis causing fetal heart failure. CASE: A multipara with two children, who died of endocardial fibroelastosis, was referred for fetal echocardiography at 20 weeks' gestation. A mildly asymmetric four-chamber view with slightly enlarged atria and no other fetal heart abnormalities suggested endocardial fibroelastosis. Doppler studies demonstrated abnormal cardiac function with extremely low atrioventricular E/A ratio, defined as the ratio between the rapid ventricular filling (E wave) and the atrial systole (A wave), and abnormal venous flow velocity waveforms establishing the diagnosis. On a repeat scan 2 weeks later, the abnormal morphologic signs suggesting endocardial fibroelastosis were more prominent, and pregnancy was terminated. CONCLUSION: When morphologic signs are not definitive, Doppler studies of the fetal heart may be useful in making the diagnosis of endocardial fibroelastosis.

Echocardiography, Doppler↗
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↗
PubMed1991

Endocardial fibroelastosis in infants with hydrops fetalis.

Endocardial fibroelastosis, defined as an endocardium in excess of 30 microns thick, was found in 10 out of 34 cases of hydrops fetalis in a review of 1589 perinatal necropsies carried out between 1976 and 1989. The infants comprised 16 cases of rhesus haemolytic disease, of whom three had endocardial fibroelastosis, and 18 cases of non-rhesus hydrops, of whom seven had endocardial fibroelastosis. Intrauterine congestive heart failure was thought to have been the probable cause of hydrops in eight of the 10 infants with endocardial fibroelastosis. None of an age matched control group without endocardial fibroelastosis had evidence of congestive cardiac failure. These observations support the hypothesis that endocardial fibroelastosis is an endocardial response to chronic prenatal myocardial stress.

Autopsy↗
PubMed1980

Congestive heart failure and endocardial fibroelastosis caused by chronic lymphocytic leukemia.

Endocardial fibroelastosis developed in the heart of a patient who had chronic lymphocytic leukemia. Leukemic infiltrates were found in the endocardial scar tissue, myocardium, coronary arteries, and other viscera. In view of the absence of any other known cause of endocardial fibroelastosis, it is postulated that endocardial fibroelastosis was caused by myocardial leukemic infiltration. This is the first reported case of endocardial fibroelastosis in a patient with chronic lymphocytic leukemia.

Endocardial Fibroelastosis↗
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↗
PubMed1988

Prenatal sonographic diagnosis of endocardial fibroelastosis secondary to aortic stenosis.

Endocardial fibroelastosis is characterized by a diffuse thickening of the left ventricular endocardium with or without other cardiac anomalies. This entity had been diagnosed prenatally previously (Bovicelli et al., 1984) at a gestational age of 36 weeks. A case of endocardial fibroelastosis due to aortic stenosis accompanied by pericardial effusion, ascites, and hydramnion, diagnosed ultrasonographically and confirmed pathologically at 21 weeks of gestation, is presented.

Adult↗
PubMed1997

Endocardial fibroelastosis in a dog.

Endocardial fibroelastosis is an uncommon congenital heart disease in dogs that may be manifested by signs of left-sided congestive heart failure. A three-month-old, male, Fila Brasileiro dog developed signs of generalised heart failure. Physical examination revealed normal temperature, ascites, and pale and cyanotic mucous membranes. The pup died just after radiography which revealed ascites, hepatomegaly, severe cardiac enlargement and pulmonary oedema. At necropsy, serosanguineous fluid in the thorax and abdomen, pulmonary oedema, right ventricular dilatation, hypertrophy and dilatation of the left ventricle, and mitral valve incompetence were observed. The histopathological examination demonstrated that the thickening of the endocardium of the left atrium and left ventricle was due to the presence of elastic and collagen fibres, although there were no signs of an inflammatory process.

Animals↗
PubMed1997

Viral infection of the myocardium in endocardial fibroelastosis. Molecular evidence for the role of mumps virus as an etiologic agent.

BACKGROUND: Endocardial fibroelastosis, previously a common disease of children, often resulted in congestive heart failure and death. Virus-induced myocarditis was the suspected first step in the pathogenesis of the disease, with enteroviruses and mumps virus considered potential causes. Direct evidence for their involvement was limited, however, and during the past two decades, a significant decline in the incidence of endocardial fibroelastosis occurred. Recently, we demonstrated polymerase chain reaction to be a rapid and sensitive method for identification of the viral genome in the myocardium of patients with myocarditis and dilated cardiomyopathy. The purpose of this study was to analyze myocardial samples of patients with endocardial fibroelastosis for the viral genome. METHODS AND RESULTS: Myocardial samples from 29 patients with autopsy-proven endocardial fibroelastosis were analyzed for viral genome (enterovirus, adenovirus, mumps, cytomegalovirus, parvovirus, influenza, herpes simplex virus) by use of polymerase chain reaction or reverse transcriptase-polymerase chain reaction. In 90% of samples, the viral genome was amplified; > 70% of the samples were positive for mumps viral RNA, while 28% amplified adenovirus. In contrast, only 1 of 65 control samples amplified a virus (enterovirus). Two regions of mumps virus were amplified: the nucleocapsid gene and the polymerase-associated protein gene. Interestingly, only 3 of the 21 samples that were positive for mumps RNA were positive with both sets of primers, indicating that the persistence of mumps virus in the myocardium may be related to the selection of defective virus mutants. CONCLUSIONS: These data suggest an etiologic role for viral infection in endocardial fibroelastosis, supporting the hypothesis that endocardial fibroelastosis is a sequela of a viral myocarditis, in particular of that due to mumps virus.

Child↗
PubMed1995

[The finding of endocardial fibroelastosis in a 20-week-old fetus].

Endocardial fibroelastosis is a rare disease that only sporadically has been diagnosed prenatally. The cases reported so far were found after the second trimester of pregnancy. We report a case of endocardial fibroelastosis found in a 20-week fetus, in whom the diagnosis was performed by echocardiography and, after voluntary interruption of pregnancy, was confirmed by necroscopy and histology. Early intrauterine detection of endocardial fibroelastosis allows to plan pregnancy, modality of delivery and a possible therapy.

Echocardiography↗