Endocardial Fibroelastosis

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). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.

Also Known As:

Networked: 110 relevant articles (0 outcomes, 6 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Cardiomyopathies (Cardiomyopathy)
2.	Heart Diseases (Heart Disease)
3.	Dilated Cardiomyopathy (Cardiomyopathy, Congestive)
4.	Myocarditis (Carditis)
5.	Aortic Valve Stenosis (Aortic Stenosis)

Experts

1.	Taylor, Glenn P: 4 articles (12/2011 - 02/2002)
2.	Hornberger, Lisa K: 4 articles (02/2011 - 02/2002)
3.	Silverman, Earl D: 3 articles (04/2017 - 02/2002)
4.	Nield, Lynne E: 3 articles (12/2011 - 02/2002)
5.	Smallhorn, Jeffrey F: 3 articles (08/2002 - 02/2002)
6.	Friehs, Ingeborg: 2 articles (12/2021 - 01/2015)
7.	Jaeggi, Edgar: 2 articles (04/2017 - 02/2011)
8.	Mullen, J Brendan M: 2 articles (08/2002 - 02/2002)
9.	Del Nido, Pedro J: 1 article (12/2021)
10.	Doulamis, Ilias P: 1 article (12/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Endocardial Fibroelastosis:

1.	AutoantibodiesIBA 08/21/2002 - "This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies. " 02/19/2002 - "Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood. " 12/01/2011 - "The observation that endocardial fibroelastosis (EFE) can result from an immune response to maternal autoantibody deposition in the fetal myocardium raises the possibility that the fetal immune system may contribute to the pathogenesis of idiopathic EFE and dilated cardiomyopathy (DCM). " 02/08/2011 - "We have previously shown that 85% of fetuses and infants with maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis suffer demise or need for transplant. " 02/08/2011 - "Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. "
2.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2019 - "In the 14 surviving children, third-degree CHB was detected in 4 children, second- to third-degree CHB in 4, corrected QT interval (QTc) prolongation in 1, atrioventricular dissociation, and junctional ectopic tachycardia in 1, complete left bundle branch block (CLBBB) with dilated cardiomyopathy (DCM) in 3, and endocardial fibroelastosis (EFE) in 1. All of the 14 surviving babies received intravenous immunoglobulin and glucocorticoids. " 02/08/2011 - "Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. " 02/08/2011 - "This study sought to evaluate the outcome of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis following intravenous gamma globulin (IVIG) and corticosteroid therapy. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2017 - "Incomplete block and endocardial fibroelastosis regressed with transplacental corticosteroid and immune globulin therapy. " 02/08/2011 - "Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. " 02/08/2011 - "This study sought to evaluate the outcome of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis following intravenous gamma globulin (IVIG) and corticosteroid therapy. "
4.	Carvedilol (Coreg)FDA LinkGeneric 09/01/2008 - "[Preliminary study on the effect of carvedilol on children with primary endocardial fibroelastosis]." 05/01/2015 - "A total of 36 patients with CHF, including 17 of endocardial fibroelastosis and 19 of dilated cardiomyopathy, were enrolled and were randomly divided into a carvedilol treatment group (group A) and a conventional treatment group (group B). "
5.	A-factor (Streptomyces)IBA 11/01/2006 - "Ninety-two percent of respondents rely on mitral valve z score, 84% on aortic valve z score, 52% on left ventricle length, 48% on the presence of antegrade ascending aorta flow, and only 32% considered significant endocardial fibroelastosis as a factor. " 08/26/1954 - "Endocardial fibroelastosis; a factor in heart disease of obscure etiology: a study of 20 autopsied cases in children and adults."
6.	Radioisotopes (Radionuclides)IBA 01/01/1986 - "Peak to peak time from the right and to the left ventricle (PPT), left ventricular ejection fraction, left ventricular peak ejection rate and left ventricular peak filling rate were measured by first pass radionuclide angiocardiography in 27 infants and children with normal heart and in 8 patients (18 studies) with endocardial fibroelastosis. " 01/01/1986 - "These results indicate that parameters obtained from the radionuclide angiocardiography are useful for evaluating cardiac performance in patients with endocardial fibroelastosis." 01/01/1986 - "Assessment of cardiac performance by first pass radionuclide angiocardiography in infants and children with normal heart and endocardial fibroelastosis."
7.	Peptides (Polypeptides)IBA 01/01/1990 - "Immunohistochemical study on human atrial natriuretic polypeptide in the ventricle of hearts with endocardial fibroelastosis." 01/01/1990 - "The presence and distribution of human atrial natriuretic polypeptide (ANP) were investigated immunohistochemically in the ventricles of hearts of 14 cases with endocardial fibroelastosis and 15 cases with noncardiac disease in children. "
8.	gamma-Globulins (gamma-Globulin)IBA 02/08/2011 - "This study sought to evaluate the outcome of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis following intravenous gamma globulin (IVIG) and corticosteroid therapy. "
9.	CollagenIBA 12/01/2020 - "Histopathology revealed moderate to severe thickening of the left ventricular endocardium, composed mostly of abundant elastic fibers and fewer collagen fibers, diagnostic for endocardial fibroelastosis. " 09/01/2013 - "Endocardial fibroelastosis (EFE) is a thickening of the endocardium by collagen and elastic fibers. " 01/01/1986 - "Besides elastic and collagen fibres, numerous smooth muscle (SM) cells were found in the thickened endocardium in 3 cases of restrictive cardiomyopathy and in 1 case of congenital endocardial fibroelastosis. " 10/01/1985 - "Alterations at 21-104 days included extensive scarring with formation of dense mature collagen with scattered fibroblasts present, grossly evident left-ventricular aneurysm, and gross and microscopic changes similar to those observed in the secondary form of endocardial fibroelastosis. " 05/01/1979 - "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). "
10.	AntibodiesIBA 04/01/2005 - "Complete fetal heart block (HB) and endocardial fibroelastosis (EFE) are known to be associated with maternal anti-Ro and anti-La antibodies. " 07/01/2023 - "Patterns of endocardial fibroelastosis without atrioventricular block in fetuses exposed to anti-Ro/SSA antibodies." 02/01/2011 - "A new presentation of neonatal lupus: 5 cases of isolated mild endocardial fibroelastosis associated with maternal Anti-SSA/Ro and Anti-SSB/La antibodies." 08/21/2002 - "Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block." 07/30/2002 - "Dilated CM was diagnosed in 22 cases, including 2 with congenital infections, 5 familial cases, 6 with endocardial fibroelastosis related to maternal anti-Ro/La antibodies, and 9 idiopathic cases. "

Therapies and Procedures

1.	Therapeutics 08/01/2010 - "To summarize and analyze the effects of treatment and prognosis of infants with endocardial fibroelastosis (EFE) in different states of the illness undergone relevant therapies, and to understand the roles of different treatments for improving the prognosis of the disease. " 03/01/1998 - "However, in multivariate analysis, only histologic evidence of endocardial fibroelastosis, clinical signs of right ventricular failure at presentation, and the need for anticoagulative therapy during follow-up, the last an expression of a severely impaired left ventricular systolic function, appeared to be significant predictors of long-term outcome. " 07/01/1995 - "Early intrauterine detection of endocardial fibroelastosis allows to plan pregnancy, modality of delivery and a possible therapy." 08/28/1969 - "[Diagnosis and therapy of endocardial fibroelastosis]." 12/01/1964 - "[ON THE DIAGNOSIS AND THERAPY OF MYOCARDOSIS WITH ENDOCARDIAL FIBROELASTOSIS]."
2.	Heart Transplantation (Grafting, Heart) 11/01/1992 - "A case is presented of constrictive endocardial fibroelastosis without other cardiac abnormality in a newborn infant who was treated successfully by orthotopic heart transplantation." 01/01/1990 - "Cardiac transplantation in a neonate with endocardial fibroelastosis." 01/01/2001 - "Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated." 04/01/1996 - "The patient with endocardial fibroelastosis died during an attempt to perform the Norwood operation (Stage I) and the other patient with ischemic dilated cardiomyopathy survived after cardiac transplantation. " 07/01/1991 - "Since March 1988, we have performed orthotopic heart transplantation in 11 infants and children with hypoplastic left heart syndrome (n = 6), cardiomyopathy (n = 4), or congenital endocardial fibroelastosis (n = 1). "
3.	Transplantation 10/31/1997 - "The hearts from infants who died or underwent transplantation appeared, on gross examination, to be enlarged, with coarse, deep ventricular trabeculations and prominent endocardial fibroelastosis. " 07/01/1992 - "Mean age at transplantation ranged from 49 years (ischemic heart disease) to 6 years (endocardial fibroelastosis). " 05/01/1991 - "Two infants had aortic stenosis with endocardial fibroelastosis, and one had extracorporeal membrane oxygenation as a bridge to transplantation. " 07/01/1998 - "Indications for transplantation included hypoplastic left-heart syndrome (n = 28), dilative cardiomyopathy (n = 4), endocardial fibroelastosis (n = 4) and other complex structural anomalies (n = 3). " 07/01/1992 - "Distribution of recipients by sex and age varied according to the indications for transplantation: males were more common among the patients transplanted for ischemic (97%) and valvular (84%) heart disease, as well as for dilated (82%) and hypertrophic (78%) cardiomyopathy, whereas the opposite was true for endocardial fibroelastosis (males constituting 21%) and cardiac tumours (25%). "
4.	Norwood Procedures 01/01/2001 - "Significant independent factors associated with greater survival benefit for the Norwood procedure versus biventricular repair included younger age at entry, lower z-score of the aortic valve and left ventricular length, higher grade of endocardial fibroelastosis, absence of important tricuspid regurgitation, and larger ascending aorta. " 04/01/1996 - "The patient with endocardial fibroelastosis died during an attempt to perform the Norwood operation (Stage I) and the other patient with ischemic dilated cardiomyopathy survived after cardiac transplantation. " 04/01/1995 - "From April 1987 to September 1993, 60 infants underwent a Norwood operation for complex congenital heart disease including hypoplastic left heart syndrome (n = 41), ventricular septal defect and subaortic stenosis with aortic arch interruption/severe coarctation (n = 7), complex single right ventricle with subaortic stenosis (n = 8), critical aortic stenosis with endocardial fibroelastosis (n = 2), and malaligned primum atrial septal defect with coarctation (n = 2). "
5.	Catheters 10/01/2022 - "The techniques for ventricular recruitment include restriction of the atrial septal defect (ASD), atrioventricular valve repair, repair of left ventricular outflow obstruction, endocardial fibroelastosis resection, an augmented pulmonary blood flow, and catheter-based interventions. " 08/01/1984 - "Only by means of transvascular endomyocardial biopsies, performed during cardiac catheterization with a 6-F guide-catheter and a Machida bioptome for light and electron microscopal examination, was differentiation achieved among six patients with endocardial fibroelastosis and six with the nonspecific histological findings of muscular hypertrophy, interstitial fibrosis and marked degenerative changes. "