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.

Vascular dilatation

Abnormal outpouching or sac-like dilatation in the wall of an atery, vein or the heart.

Total: 5

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PMID (PMCID)
20303141	FEMALE	Infant, Newborn
	Mitogenic cardiomyopathy: a lethal neonatal familial dilated cardiomyopathy characterized by myocyte hyperplasia and proliferation.
	Chang KT, Taylor GP, Meschino WS, Kantor PF, Cutz E. Hum Pathol. 2010;41(7):1002-8.
	Autopsy revealed congestive cardiac failure and enlarged, dilated hearts with ventricular dilatation more pronounced than atrial dilatation, and endocardial fibroelastosis.
20303141	FEMALE	Infant, Newborn
	Mitogenic cardiomyopathy: a lethal neonatal familial dilated cardiomyopathy characterized by myocyte hyperplasia and proliferation.
	Chang KT, Taylor GP, Meschino WS, Kantor PF, Cutz E. Hum Pathol. 2010;41(7):1002-8.
	Autopsy revealed congestive cardiac failure and enlarged, dilated hearts with ventricular dilatation more pronounced than atrial dilatation, and endocardial fibroelastosis.
16523519	MIXED_SAMPLE
	Fetus with two identical reciprocal translocations: description of a rare complication of consanguinity.
	Martinet D, Vial Y, Thonney F, Beckmann JS, Meagher-Villemure K, Unger S. Am J Med Genet A. 2006;140(7):769-74.
	At autopsy, the following anomalies were found: Pierre-Robin sequence, hypoplasia of the right ventricle with muscular hypertrophy, and endocardial fibroelastosis, hypoplastic lungs, dysplastic left kidney, bilateral pelvicalyceal dilatation, central nervous system periventricular heterotopias and right sided club foot.
9185226	FEMALE
	Autopsy findings in the Wolcott-Rallison syndrome.
	Thornton CM, Carson DJ, Stewart FJ. Pediatr Pathol Lab Med. 1997;17(3):487-96.
	The heart was enlarged with mitral value dysplasia and stenosis, left atrial dilatation, left ventricular hypertrophy, and endocardial fibroelastosis.
2969084	MIXED_SAMPLE	Infant, Newborn
	Fetal echocardiographic signs of congenital endocardial fibroelastosis.
	Veille JC, Sivakoff M. Obstet Gynecol. 1988;72(2):219-22.
	Endocardial fibroelastosis is characterized by a porcelain-like thickening of the endocardium, resulting in a marked increase in echodensity of the endocardium, as well as ventricular dilatation and aortic atresia.