• 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 thickening extends to the LA, right ventricle, and right atrium. (medscape.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)
  • Failure of the tricuspid valve to close sufficiently upon contraction of the right ventricle, causing blood to regurgitate (flow backward) into the right atrium. (beds.ac.uk)
  • Note the thickened endocardium (endocardial fibroelastosis) within the left atrium. (ipccc.net)
  • De-oxygenated blood enters the right atrium of the heart and flows into the right ventricle where it is pumped through the pulmonary arteries to the lungs. (wikidoc.org)
  • Pulmonary veins return the now oxygen-rich blood to the heart, where it enters the left atrium before flowing into the left ventricle. (wikidoc.org)
  • left atrium , left ventricle , right atrium and right ventricle . (wikidoc.org)
  • BACKGROUND: Atrioventricular septal defect (AVSD) and double outlet right ventricle (DORV) with normally related great arteries and normal ventricular sizes are associated with a good long-term prognosis after biventricular (BV) repair. (mcmaster.ca)
  • 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)
  • Endomyocardial fibrosis (EMF) occurs commonly in tropical regions and affects both the left and right ventricles. (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)
  • Rats injected with ISO exhibited severe interstitial cardiac fibrosis and perivascular fibrosis, decreased left ventricular ejection fraction, and increased NOX activity. (dovepress.com)
  • TMZ treatment mitigated cardiac fibrosis, ameliorated left ventricular dysfunction, and reduced NOX activity. (dovepress.com)
  • 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)
  • Endocardial fibroelastosis (EFE) is characterized by a diffuse white fibrous tissue lining the endocardium. (nih.gov)
  • A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). (childrensmercy.org)
  • Rarely, left ventricular dysfunction and symptomatic congestive heart failure occur unless stenosis is reduced and/or insufficiency is relieved. (medscape.com)
  • Endocardial fibroelastosis (EFE), which occurs in infants and children, affects only the left ventricle. (msdmanuals.com)
  • Severe obstruction in utero may lead to variable degrees of left-sided heart hypoplasia, endomyocardial fibroelastosis, reduced ventricular function, and significant mitral valve insufficiency. (medscape.com)
  • Rare congenital cardiomyopathies characterized by the lack of left ventricular myocardium compaction. (mcw.edu)
  • [ 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)
  • For this reason, most patients have left myocardial thickening (hypertrophy). (wikipedia.org)
  • Primary RCMs are rare myocardial diseases and are defined by restrictive ventricular physiology in the presence of normal or reduced diastolic volumes of one or both ventricles, normal or reduced systolic volumes, and normal ventricular wall thickness, with significant atrial dilation. (mhmedical.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)
  • The noncompaction results in numerous prominent trabeculations and a loose myocardial meshwork (spongy myocardium) in the LEFT VENTRICLE. (mcw.edu)
  • The right and left atria and the right ventricle are markedly enlarged and hypertrophied, with minimal or no endocardial sclerosis. (medscape.com)
  • Surgical repair or replacement of an aortic valve is primarily reserved for patients in whom balloon valvuloplasty has failed with severe stenosis or have significant valve insufficiency in association with progressive left ventricular dilation or deterioration of left ventricular systolic function (see Treatment and Management). (medscape.com)
  • Secondary calcification of the valve is extremely rare, and at times, the aortic valve anulus may also be underdeveloped or hypoplastic in association with mitral and left ventricular hypoplasia, adding to the severity of left ventricular outflow tract (LVOT) obstruction. (medscape.com)
  • Normal RV functional Overload LV-This may be seen occasionally in LV dysfunction in conditions like myocarditis, endocardial fibroelastosis or the dilated form of critical aortic stenosis. (usdoc.co.il)
  • Echocardiograms demonstrated vigorous systolic function but substantial diastolic dysfunction of the left ventricle in all. (nih.gov)
  • This chapter provides an overview of restrictive heart diseases, conditions characterized by diastolic dysfunction in the presence of elevated left ventricular filling pressures and limited increase in volume. (mhmedical.com)
  • Restrictive cardiomyopathy (RCM) is a rare disorder in children that is characterized by restrictive filling and reduced diastolic volume of one or both ventricles with normal or near-normal systolic function and wall thickness. (medscape.com)
  • Heterogeneous clinical features include diminished systolic function sometimes associated with left ventricular dilation, that presents either neonatally or progressively. (mcw.edu)
  • in rare cases, however, a large adolescent patient may require transesophageal echocardiography to clearly delineate the left ventricular outflow tract and to detail the valve anatomy. (medscape.com)
  • This is probably related to decreased compliance of the ventricle and ultimately results in the development of atrial dilatation. (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)
  • The myocardium is dilated, reducing the systolic pump of the ventricles. (wikipedia.org)
  • Mutations in the alpha-dystrobrevin gene are associated with Autosomal Dominant type of Noncompaction of the Left Ventricular Myocardium. (bvsalud.org)
  • Mutations in the tafazzin gene are associated with X-Linked Isolated Noncompaction of the Left Ventricular Myocardium. (bvsalud.org)
  • This stenosis causes mild to severe obstruction of the left ventricular outflow that may be associated with other left heart obstructive lesions, varying degrees of left heart hypoplasia, or extracardiac malformations, including genetic disorders. (medscape.com)
  • Either type may be diffuse or nondiffuse (when the disorder affects only one ventricle or part of one ventricle unevenly). (msdmanuals.com)
  • Fetal echocardiographic parameters comparing BV repair versus single ventricle (SV) palliation were obtained, including the presence or absence of an apex-forming bLV was recorded. (mcmaster.ca)
  • An early event in fetal life is believed to result in dilated endocardial fibroelastosis, which later morphoses into a contracted type. (medscape.com)
  • RCM may manifest as a solitary abnormality, although restrictive filling patterns of the left ventricle can also be seen in patients with dilated or hypertrophic cardiomyopathy. (medscape.com)
  • Shape Analysis and Computational Fluid Simulations to Assess Feline Left Atrial Function and Thrombogenesis. (cardiacmorphology.com)
  • In this image, a stenotic mitral valve is viewed from the left atrial vestibule. (ipccc.net)
  • While humans, as well as other vertebrates , have a closed cardiovascular system (meaning that the blood never leaves the network of arteries, veins and capillaries), some invertebrate groups have an open cardiovascular system. (wikidoc.org)
  • The cardiovascular systems of humans is closed, meaning that the blood never leaves the system of blood vessels . (wikidoc.org)
  • The ventricular muscle twitches randomly rather than contracting in a co-ordinated fashion (from the apex of the heart to the outflow of the ventricles), and so the ventricles fail to pump blood around the body - because of this, it is classified as a cardiac arrest rhythm, and patients in V-fib should be treated with cardiopulmonary resuscitation (CPR) and prompt defibrillation . (mdwiki.org)
  • Mild endocardial brightening of the anterior septum, anterior wall, or papillary muscles was present in two. (nih.gov)
  • Mild-to-moderate degrees of obstruction are usually well tolerated, with minimal hypertrophy and normal left ventricular function. (medscape.com)
  • It aims to bring specialized services to this unique population by offering exercise rehabilitation, mental health and behavioral care, nutrition and dietary needs, and patient-family education to those with a single ventricle and Fontan circulation. (chop.edu)
  • Left untreated, ventricular fibrillation is rapidly fatal as the vital organs of the body, including the heart, are starved of oxygen, and as a result patients in this rhythm will not be conscious or responsive to stimuli. (mdwiki.org)
  • Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year experience. (chop.edu)
  • Endocardial Fibroelastosis" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (childrensmercy.org)
  • Restrictive cardiomyopathy can affect one or both ventricles. (msdmanuals.com)
  • 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)
  • As leader of the Single Ventricle Survivorship Clinic, Dr. Rychik is focused on improving therapies. (chop.edu)
  • He's studying the long-term effects of current management strategies for single ventricle conditions. (chop.edu)
  • The endeavor builds upon the Single Ventricle Survivorship Clinic. (chop.edu)
  • 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)