Endocardial fibroelastosis

Endomyocardial fibroelastosis is a cause of unexplained childhood cardiac insufficiency. It results from diffuse thickening of the endocardium leading to dilated myocardiopathy in the majority of cases and restrictive myocardiopathy in rare cases. It may occur as a primary disorder or may be secondary to another cardiac malformation, notably aortic stenosis or atresia.

Cardiomyopathy

A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.


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PMID (PMCID)
24969939
 MIXED_SAMPLE Infant
[Congenital atresia of left main coronary artery in 4 children: case report and literature review].
Xiao Y, Han L, Jin M, Ding W.
Zhonghua Er Ke Za Zhi. 2014;52(5):383-6.
CLMCA-A is not rare, its clinical manifestations should be differentiated from those of cardiomyopathy, endocardial fibroelastosis, congenital valvular disease and abnormal left coronary artery originating from pulmonary artery etc.
24147243
(3799713)
 OTHER
Resolution of neonatal hypertrophic cardiomyopathy presumed secondary to acquired maternal ribonucleoprotein and smith autoantibodies.
Shah A, Winrow A, Fulljames R, Naqvi N, Bansal RA, Bansal AS.
AJP Rep. 2013;3(2):91-6.
Previously reported cases of neonatal cardiomyopathy with endocardial fibroelastosis have been ascribed to the transplacental transfer of maternal Sjogrens Syndrome (SS) A (Ro) and Sjogrens Syndrome (SS) B (La) antibodies and have been more severe and persistent compared with our patient.
17926085
 MALE Infant, Newborn
Neonatal atrial fibrillation after surgical repair of tracheoesophageal fistula with esophageal atresia.
Mainzer G, Khoury A, Gelernter-Yaniv L, Lorber A.
Pediatr Cardiol. 2008;29(1):150-2.
A report of 35 pediatric patients in a single center over a period of 22 years identified atrial fibrillation in children with a variety of ailments including congenital cardiac anomalies before and after corrective surgery, rheumatic valve disease, Marfan's syndrome with mitral regurgitation, infective endocarditis, cardiomyopathy, endocardial fibroelastosis, paroxysmal atrial tachycardia of infants, and cardiac tumors [2].
17346634
 MALE Infant
X-linked cardiomyopathy presenting as contracted endocardial fibroelastosis.
Sjoberg G, Chow CW, Cooper S, Weintraub RG.
J Heart Lung Transplant. 2007;26(3):293-5.
X-linked cardiomyopathy presenting as contracted endocardial fibroelastosis.
16377939
 FEMALE Infant, Newborn
Newborn infant with maternal anti-SSA antibody-induced complete heart block accompanying cardiomyopathy.
Iida M, Inamura N, Takeuchi M.
Circ J. 2006;70(1):147-9.
Autopsy revealed left ventricular cardiomyopathy with endocardial fibroelastosis.
16797345
 FEMALE
Unusual case of an 18-year-old heart transplant recipient with endocardial fibroelastosis.
Fuchs U, Zittermann A, Schulz U, Tenderich G, Raute-Kreinsen U, Minami K, Korfer R.
Transplant Proc. 2006;38(5):1511-3.
We concluded that endomyocardial fibroelastosis has to be considered a cause of heart failure in young adults with unclear cardiomyopathy.
9252790
 MIXED_SAMPLE Infant
Combined 3-methylglutaconic and 3-hydroxy-3-methylglutaric aciduria with endocardial fibroelastosis and dilatative cardiomyopathy in male and female siblings with partial deficiency of complex II/III in fibroblasts.
Ruesch S, Krahenbuhl S, Kleinle S, Liechti-Gallati S, Schaffner T, Wermuth B, Weber J, Wiesmann UN.
Enzyme Protein. 1996;49(5-6):321-9.
Combined 3-methylglutaconic and 3-hydroxy-3-methylglutaric aciduria with endocardial fibroelastosis and dilatative cardiomyopathy in male and female siblings with partial deficiency of complex II/III in fibroblasts.
2033908
 OTHER Infant
[Heart transplantation in infants--a new option].
Netz H, Bauer J, Kreuder J, Moosdorf R, Hehrlein FW.
Klin Padiatr. 1991;203(2):72-6.
Since march 1988 we have performed an orthotopic heart transplantation in 10 pediatric patients: 5 infants (2-16 years of age) with congestive cardiomyopathy, 1 newborn with endocardial fibroelastosis, 3 newborns with hypoplastic left heart syndrome, and finally 1 infant at the age of 2 years with AV-canal and hypoplastic left ventricle.
3681864
 FEMALE Adult
Peripartum congestive cardiomyopathy and endocardial fibroelastosis associated with ritodrine treatment. A case report.
Edoute Y, Blumenfeld Z, Bronstein M, Aharoni L, Moskowitz M.
J Reprod Med. 1987;32(10):793-7.
Congestive cardiomyopathy from endocardial fibroelastosis occurred in a 24-year-old primigravida with a twin gestation and preeclampsia.
6612319
 MALE Child
Endocardial fibroelastosis associated with fatal adriamycin (doxorubicin) cardiomyopathy during treatment of Ewing's sarcoma.
Conroy B, Guthrie W.
Scott Med J. 1983;28(3):295-9.
Endocardial fibroelastosis associated with fatal adriamycin (doxorubicin) cardiomyopathy during treatment of Ewing's sarcoma.
148030
 MIXED_SAMPLE Child
Familial nonobstructive cardiomyopathy with endocardial fibroelastosis beyond infancy.
Singh A, Doyle EF, Danilowicz DA, Finegold MJ.
Pediatrics. 1978;61(3):410-6.
Probably the endocardial fibroelastosis was secondary to a familial nonobstructive cardiomyopathy.