Dilated cardiomyopathyPathologic forms of primary endocardial fibroelastosisFibrosisCardiomyopathy and endocardial fibroelastosisStructural anomaly of the heartCardiacSecondaryMyocardiumCongenital heart dPathophysiologyPrimaryMyocardialEndocardiumDiagnosisSyndromeClinicalMarkedly enlargedVentricleFatal1943MildFetalVentricularSymptomsChildrenRareFormShowsArticleLayerReferenceCells
Dilated cardiomyopathy3
- 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)
- 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)
- Mutations in the muscle LIM protein and alpha-actinin-2 genes in dilated cardiomyopathy and endocardial fibroelastosis. (dcmproject.com)
Pathologic forms of primary endocardial fibroelastosis2
- [ 2 ] aortic stenosis, or atresia.The two pathologic forms of primary endocardial fibroelastosis are dilated, which is most common, and contracted. (medscape.com)
- The 2 pathologic forms of primary endocardial fibroelastosis include dilated, which is most common, and contracted. (medscape.com)
Fibrosis4
- No study was thought to be diagnostic of endocardial fibrosis. (nih.gov)
- In patients with hypereosinophilic syndrome and extensive endocardial fibrosis, thrombus can form in the apices and inflow tracts ventricles compromising the size of the ventricular cavities. (msdmanuals.com)
- 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)
- 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)
Cardiomyopathy and endocardial fibroelastosis1
- The antibodies are also postulated to affect myocardial cells resulting in cardiomyopathy and endocardial fibroelastosis. (ccasociety.org)
Structural anomaly of the heart1
- Primary endocardial fibroelastosis is not associated with any significant structural anomaly of the heart. (medscape.com)
Cardiac5
- 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)
- 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 single group remains unassociated with other cardiac abnormalities, and this is termed "primary" endocardial fibroelastosis. (jamanetwork.com)
- Maredia N, English K, Greenwood J. Assessment of endocardial fibroelastosis by cardiac MRI. (medscape.com)
- Secondary endocardial fibroelastosis, associated with cardiac malformations, is attributed to the cardiac hypertrophy and consequent imbalance in the myocardial oxygen supply-demand relationship. (medscape.com)
Secondary5
- 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)
- Secondary endocardial fibroelastosis is associated with other congenital heart diseases. (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)
- Andersen and Kelly, 2 in 1956, showed that many children with congenital heart disease develop endocardial fibroelastosis, apparently secondary to hemodynamic factors or anoxia in the malformed heart. (jamanetwork.com)
- Achiron R, Malinger G, Zaidel L, Zakut H. Prenatal sonographic diagnosis of endocardial fibroelastosis secondary to aortic stenosis. (medscape.com)
Myocardium2
- 3 The etiology of primary endocardial fibroelastosis is not certain, but it has been suggested that this lesion is a genetically determined developmental anomaly, possibly due to an enzymatic defect of the myocardium. (jamanetwork.com)
- Viral infection of the myocardium in endocardial fibroelastosis. (medscape.com)
Congenital heart d1
- 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)
Pathophysiology1
- 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)
Primary4
- Primary endocardial fibroelastosis prognosis is relatively poor, although the condition is not universally fatal. (medscape.com)
- 1 The occurrence of primary endocardial fibroelastosis in siblings is rare. (jamanetwork.com)
- Primary endocardial fibroelastosis: an underappreciated cause of cardiomyopathy in children. (medscape.com)
- The less common contracted type of primary endocardial fibroelastosis is associated with a relatively hypoplastic or normal LV size. (medscape.com)
Myocardial2
- [ 2 ] Endocardial fibroelastosis is characterized by diffuse endocardial thickening and myocardial dysfunction. (medscape.com)
- On MRI EFE manifested at the endocardial surface as a rim of hypointense signal in the perfusion sequences and as a rim of hyperintense signal in the myocardial delayed-enhancement sequences. (nih.gov)
Endocardium4
- 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)
- 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)
- Endocardial fibroelastosis (EFE) is characterized by a diffuse white fibrous tissue lining the endocardium. (nih.gov)
- Note the thickened endocardium (endocardial fibroelastosis) within the left atrium. (ipccc.net)
Diagnosis3
- Risk of endocardial fibroelastosis (EFE) in subsequent pregnancies is 3-5% and warrants fetal echocardiography for early diagnosis. (medscape.com)
- Contracted form of endocardial fibroelastosis: angiographic diagnosis and necropsy confirmation. (medscape.com)
- Prenatal diagnosis of endocardial fibroelastosis. (medscape.com)
Syndrome1
- Syndrome that is characterized by communicating hydrocephalus, endocardial fibroelastosis and congenital cataracts. (nih.gov)
Clinical1
- Endocardial fibroelastosis: a clinical and anatomic study of 47 patients with emphasis on its relationship to mitral insufficiency. (medscape.com)
Markedly enlarged1
- The right and left atria and the right ventricle are markedly enlarged and hypertrophied, with minimal or no endocardial sclerosis. (medscape.com)
Ventricle3
- Endocardial thickening extends to the LA, right ventricle, and right atrium. (medscape.com)
- Endocardial fibroelastosis (EFE), which occurs in infants and children, affects only the left ventricle. (msdmanuals.com)
- Endocardial fibroelastosis (a fatal disease, enlarged left atrium and ventricle), congenital keratoconjunctivitis (dry eye), problem with the eyelid similar to cherry eye. (thepetwiki.com)
Fatal1
- I hope my story gives other people hope in similar situations and hope it shows that endocardial fibroelastosis is not always fatal and there is some hope for others who have it. (myheart.org.uk)
19432
- 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)
Mild1
- Mild endocardial brightening of the anterior septum, anterior wall, or papillary muscles was present in two. (nih.gov)
Fetal2
- Endocardial fibroelastosis was first reported by Kreysig, in 1816, under the misnomer of fetal endocarditis. (jamanetwork.com)
- An early event in fetal life is believed to result in dilated endocardial fibroelastosis, which later morphoses into a contracted type. (medscape.com)
Ventricular2
- Parameters such as ratio of valve annuli, ventricular end-diastolic dimensions, degree of valve regurgitation, and the presence of endocardial fibroelastosis were not too predictive of outcome. (mcmaster.ca)
- Las características clínicas son heterogéneas e incluyen la disminución de la función sistólica, a veces asociada con dilatación ventricular izquierda, que se presenta ya en el periodo neonatal o progresivamente. (bvsalud.org)
Symptoms1
- 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)
Children2
- Endocardial fibroelastosis (EFE) is a rare heart disorder usually occurring in children two years old and younger. (wikipedia.org)
- And children populations, extremities inspect each extremity for symmetry of nipple placement and farmacia viagra caja cuesta cunto una de en la is being treated with oral doxycycline. (lowerbricktown.com)
Rare1
- Once regarded as a common cause of unexplained heart failure, endocardial fibroelastosis is now considered rare. (medscape.com)
Form1
- Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis. (childrensmercy.org)
Shows1
- This graph shows the total number of publications written about "Endocardial Fibroelastosis" by people in this website by year, and whether "Endocardial Fibroelastosis" was a major or minor topic of these publications. (childrensmercy.org)
Article1
- Please submit any comments about the article Endocardial Fibroelastosis The StatPearls content team will be working hard to evaluate the comment and make corrections when appropriate. (statpearls.com)
Layer1
- On autopsy, most of these patients' hearts showed the thickened endocardial layer noted above. (wikipedia.org)
Reference1
- Chen S, Thompson MW, Rose V. Endocardial fibroelastosis: family studies with special reference to counseling. (medscape.com)
Cells1
- A report suggests that the epicardium-derived mesenchymal cells could be the origin of the endocardial fibroelastosis fibroblasts. (medscape.com)