Endocardial fibroelastosis. Medical search. Frequent questions

Fibroelastosis is strongly seen as a primary cause of restrictive cardiomyopathy in children, along with cardiac amyloidosis, which is more commonly seen in progressive multiple myeloma patients and the elderly. (wikipedia.org)
Primary endocardial fibroelastosis: an underappreciated cause of cardiomyopathy in children. (medscape.com)
Arya SO, Karpawich PP, Gupta P, Buddhe S, Singh HR, Hussein Y. Primary endocardial fibroelastosis presenting in a young child as incessant ventricular tachycardia and dilated cardiomyopathy. (medscape.com)
However, a study has highlighted that 25% of children who were transplanted for dilated cardiomyopathy demonstrated significant endocardial fibroelastosis on histopathology of the explanted hearts. (medscape.com)
The diagnoses were Brugada syndrome (n=4), ischemic heart disease (n=3), dilated cardiomyopathy (n=2), hypertrophic cardiomyopathy (n=1), short-coupled variant of torsades de pointes (n=1), endocardial fibroelastosis (n=1) and idiopathic VF (n=1). (revespcardiol.org)
Cardiomyopathy - A weak heart muscle usually associated with enlargement of the heart (usually dilated with variable myocardial hypertrophy, sometimes with left ventricular noncompaction and/or endocardial fibroelastosis). (barthsyndrome.ca)
The diastolic dysfunction may be observed in myocardial (restrictive cardiomyopathy [RCM] and phenocopies), endocardial/endomyocardial (fibrosis, elastosis), and pericardial (constriction, effusion) diseases (see Fuster and Hurst's Central Illustration). (mhmedical.com)

This was thought to be a disease affecting both the heart muscle and the endocardium and it was given various names such as: idiopathic hypertrophy of the heart, endocardial sclerosis, cardiac enlargement of unknown cause, etc. (wikipedia.org)
A diagnosis of endocardial fibroelastosis in utero using fetal echocardiography may be made on the basis of increased echodensity of the endocardium and poor contractility of the ventricle. (nih.gov)
Endocardial fibroelastosis (EFE) refers to a pronounced, diffuse thickening of the ventricular endocardium and presents as unexplained heart failure in infants and children. (medscape.com)
The underlying pathophysiology of endocardial fibroelastosis (EFE) is believed to be deposition of acellular fibrocartilagenous tissue in the subendothelial layer of the endocardium predominantly involving the inflow tracts, apices of either left or both ventricles. (medscape.com)
Dilated endocardial fibroelastosis is characterized by a markedly enlarged globular heart, mainly involving the left ventricle (LV) and left atrium (LA). The LV endocardium is opaque, glistening, milky white, and diffusely thickened to about 1-2 mm. (medscape.com)
The endocardium showed prominent endocardial fibroelastosis, particularly in sections obtained from the right side of the heart. (netlify.app)
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Tropical EMF and Löeffler endocarditis should be distinguished from endocardial fibroelastosis, which is characterized by cartilaginous thickening of the mural endocardium, chiefly of the left ventricle. (medscape.com)

[ 2 ] Endocardial fibroelastosis is characterized by diffuse endocardial thickening and myocardial dysfunction. (medscape.com)
Endocardial thickening or myocardial infiltration (sometimes with death of myocytes, papillary muscle infiltration, compensatory myocardial hypertrophy, and fibrosis) may occur in one, typically the left, or both ventricles. (msdmanuals.com)
The endocardial and myocardial cells originate from populations of mesodermal cells that migrate from the midbrain-hindbrain boundary to form the linear heart tube [ 1 , 2 ]. (biomedcentral.com)

Maredia N, English K, Greenwood J. Assessment of endocardial fibroelastosis by cardiac MRI. (medscape.com)
Endocardial fibroelastosis (EFE) is a rare cardiac condition characterized by excessive endocardial thickening secondary to fibroelastic tissues that commonly present in infants and young children. (nih.gov)
Most of endocardial fibroelastosis cases are secondary forms, which occur in conjunction with other cardiac diseases. (nih.gov)

In light of recent advancements in understanding pathophysiology, several new data have revealed compelling evidence that abnormal endothelial-to-mesenchymal transition is the root cause of endocardial fibroelastosis. (nih.gov)
Endocardial fibroelastosis is caused by aberrant endothelial to mesenchymal transition. (nih.gov)

In endomyocardial fibrosis (EMF), the underlying process produces patchy fibrosis of the endocardial surface of the heart, leading to reduced compliance and, ultimately, restrictive physiology as the endomyocardial surface becomes more generally involved. (medscape.com)
Restrictive endomyocardial diseases include endomyocardial fibrosis of right, left, or both ventricles, frequently with involvement of valves, Hedinger syndrome of the right-sided heart valves in patients with neuroendocrine tumors and carcinoid syndrome, as well as endocardial fibroelastosis associated with congenital heart anomalies. (mhmedical.com)

Achiron R, Malinger G, Zaidel L, Zakut H. Prenatal sonographic diagnosis of endocardial fibroelastosis secondary to aortic stenosis. (medscape.com)
[ 2 ] aortic stenosis, or atresia.The two pathologic forms of primary endocardial fibroelastosis are dilated, which is most common, and contracted. (medscape.com)

citation needed] The term "endocardial fibroelastosis" was introduced by Weinberg and Himmelfarb in 1943. (wikipedia.org)
The term endocardial fibroelastosis was introduced by Weinberg and Himmelfarb in 1943. (medscape.com)
In 1943, the endocardial fibroelastosis term was coined by Weinberg et al. (nih.gov)

Viral infection of the myocardium in endocardial fibroelastosis. (medscape.com)
The endocardial thickening is believed to be caused by persistent and increased wall tension in the ventricles, possibly secondary to damaged myocardium, mitral regurgitation, or both. (medscape.com)

Fibroblasts in an endocardial fibroelastosis disease model mainly originate from mesenchymal derivatives of epicardium. (medscape.com)
A report suggests that the epicardium-derived mesenchymal cells could be the origin of the endocardial fibroelastosis fibroblasts. (medscape.com)

Secondary endocardial fibroelastosis is associated with other congenital heart diseases. (medscape.com)
Careful assessment of the heart for any associated congenital heart disease including coronary artery anomalies is essential before making a diagnosis of primary endocardial fibroelastosis. (medscape.com)

Risk of endocardial fibroelastosis in subsequent pregnancies is 3-5% and warrants fetal echocardiography for early diagnosis. (medscape.com)
We describe a case of very early diagnosis of fibroelastosis and aortic valve stenosis observed in utero at 14 weeks' gestation by transvaginal echocardiography. (nih.gov)

Primary endocardial fibroelastosis is not associated with any significant structural anomaly of the heart. (medscape.com)
Primary endocardial fibroelastosis prognosis is relatively poor, although the condition is not universally fatal. (medscape.com)
Primary endocardial fibroelastosis. (bmj.com)

Use endocardial fibroelastosis, malignant effusions. (mynarch.net)
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Endocardial fibrosis principally involves the apices of the right and left ventricles and may affect the atrioventricular valves mainly by tethering the papillary muscles, leading to tricuspid and mitral regurgitation. (medscape.com)

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Keller SB, Cohen J, Moon-Grady A, Cuneo B, Paul E, Coll AC, Campbell M, Srivastava S. Patterns of endocardial fibroelastosis without atrioventricular block in fetuses exposed to anti-Ro/SSA antibodies. (ucdenver.edu)

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy: A case series. (medscape.com)

Endocardial fibroelastosis has been associated with poor prognosis and outcomes. (nih.gov)

In 1957 Black-Schaffer proposed a unitary explanation that stress on the ventricle, of any kind, may trigger the endocardial reaction, so that all EFE could be thought of as secondary. (wikipedia.org)

Endocardial fibroelastosis (EFE) is primarily a disease of infants and children, but can rarely present in adulthood as well. (nih.gov)
Endocardial fibroelastosis (EFE), which occurs in infants and children, affects only the left ventricle. (msdmanuals.com)

Endocardial fibroelastosis (EFE) is a rare heart disorder usually occurring in children two years old and younger. (wikipedia.org)
Endocardial fibroelastosis of the left ventricle affects right ventricular performance in fetuses with hypoplastic left heart syndrome: a prospective study using M-mode, PW- and tissue Doppler techniques. (medscape.com)
Once regarded as a common cause of unexplained heart failure, endocardial fibroelastosis is now considered rare. (medscape.com)
Other signs and symptoms may include increased levels of certain organic acids in the urine and blood (such as 3-methylglutaconic acid), and increased thickness of the left ventricle of the heart due to endocardial fibroelastosis, which can cause potential heart issues. (nih.gov)

Endocardial fibroelastosis: a clinical and anatomic study of 47 patients with emphasis on its relationship to mitral insufficiency. (medscape.com)

EFE had quickly become the name of a disease, and it continues to be used by many physicians in this way, though many patients with identical symptoms do not have the endocardial reaction of EFE. (wikipedia.org)

MR findings of endocardial fibroelastosis in children. (medscape.com)

ventricular cavum is indicated by the red curve) and transmural homogeneous perfusion (no significant differences between the septal positions: endocardial layer indicated by the blue curve and outer layer by the yellow curve). (bmj.com)

Chen S, Thompson MW, Rose V. Endocardial fibroelastosis: family studies with special reference to counseling. (medscape.com)

On autopsy, most of these patients' hearts showed the thickened endocardial layer noted above. (wikipedia.org)

Despite the fact that generally sound, the Javanese is defenseless to endocardial fibroelastosis and bulge of the cranial sternum, a hereditary imperfection ordinarily found in varieties identified with the Siamese. (catsaesthetic.com)

Anatomy2

Diseases16

Information Science1

Named Groups1

Cardiomyopathy7

Endocardium8

Myocardial3

Cardiac3

Endothelial-to-mesenchymal transition2

Endomyocardial2

Aortic stenosis2

19433

Myocardium2

Fibroblasts2

Congenital heart2

Diagnosis2

Primary3

Malignant2

Fibrosis1

Barth1

Atrioventricular2

Pulmonary1

Prognosis1

Secondary1

Infants2

Heart4

Clinical1

Symptoms1

Children1

Significant1

Reference1

Layer1

Generally1