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Results for “ENDOCARDIAL FIBROELASTOSIS”

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At least 127 records · Page 7Linked to original sources
PubMed1999

Congenital endocardial fibroelastosis in a dog.

A 9-week-old Weimaraner was tentatively diagnosed clinically with congenital left-sided heart failure. On necropsy, diffuse fibroplasia over the left ventricular endocardium; small, deformed papillary muscles; and pulmonary congestion were evident. Microscopically, the fibroplasia extended into the myocardium and cardiomyocytes, and Purkinje fibers were degenerated, consistent with congenital endocardial fibroelastosis.

Animals↗
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↗
PubMed1976

[A rare case of endocardial fibroelastosis in the left atrium simulating mitral stenosis (author's transl)].

A rare case of primary endocardial fibroelastosis, involving only the left atrium, simulating mitral stenosis is reported; the clinical and hemodynamic features which led to the diagnosis of mitral stenosis are described. The authors discuss the data upon which a differential diagnosis between the two similar diseases can be established. The suggest that isolated fibroelastosis of the left atrium could have been developed slowly because of ipoxia of the left atrium for agenesia of the circumflex coronary artery. An operation of endotelial decortication of the left atrium is described.

Child, Preschool↗
PubMedDate not supplied

Endocardial fibroelastosis in L-transposition of the great arteries with Ebstein's anomaly: revisited.

Ebstein's anomaly is a congenital deformity of the tricuspid valve consisting mainly of leaflet malinsertion. Clinical presentation varies from asymptomatic patients to those with congestive heart failure secondary to significant valvular regurgitation and low right ventricular output. We report here the case of an infant with a diagnosis of corrected transposition of the great arteries and Ebstein's deformity of the left-sided tricuspid valve who developed pulmonary hypertension and endocardial fibroelastosis, two unusual associations with this lesion. We also discuss the pathophysiology of this association and related literature.

Ebstein Anomaly↗
PubMed2006

Sarcomere protein gene mutations and inherited heart disease: a beta-cardiac myosin heavy chain mutation causing endocardial fibroelastosis and heart failure.

Inherited human cardiomyopathies often lead to heart failure. A common feature of these conditions is that affected individuals can express the disease causing mutations for many years without showing clinical signs of the disease. Previous studies have demonstrated that sarcomere protein gene mutations can cause either dilated cardiomyopathy or hypertrophic cardiomyopathy. Here we demonstrate that the Arg442His missense mutation in beta-cardiac myosin heavy chain (betaMHC) causes dilated cardiomyopathy, endocardial fibroelastosis and heart failure at a very early age. Using standard genetic engineering tools we and others have made murine models by introducing human disease causing mutations into mice. The central hypothesis of these studies has been that by identifying the pathophysiological pathways activated by these mutations we can define enzymatic activities that are modified during the disease process and which may be involved in pathways that involve more common forms of cardiac disease. Murine models bearing different mutant myosins are being used to address whether each disease causing mutant betaMHC activates the same or different cellular pathways. Dissecting the molecular pathways modulated by mutations in sarcomere protein genes as well as other genes has already demonstrated that there are multiple pathways leading to cardiac remodelling and heart failure. Defining the mechanisms by which mutations in the same genes activate different cellular pathways remains an important question.

Adult↗
PubMedDate not supplied

Reduced left ventricular size and endocardial fibroelastosis as correlates of mortality in newborns and young infants with severe aortic valve stenosis.

30 patients with severe aortic valve stenosis presented in severe congestive heart failure within the first 2 months of life. In 25 of them, left ventricular volume and contractility were assessed; five of them had a left ventricle of normal size, in 11 left ventricular size was diminished, and in nine patients it was enlarged. Eleven of the infants had extensive endocardial fibroelastosis (EFE) evidenced angiographically by myocardial sinusoids in ten of them and established at autopsy in six. The presence of EFE correlated with the size of the left ventricle; eight of 11 with a small left ventricle, two of five with a normal-sized left ventricle, and one of nine with an enlarged left ventricle displayed EFE. The severe depression of left ventricular function associated with EFE was documented by left ventricular volume determinations on exclusion of the myocardial sinusoids. Of 30 patients, 12 (including eight of 26 who underwent surgery) did not survive. Mortality, severity, and early onset of symptoms were associated mainly with small size of the left ventricle and with the severe left ventricular dysfunction associated with EFE.

Aortic Valve Stenosis↗