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.

Aortic valve stenosis

The presence of a stenosis (narrowing) of the aortic valve.


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PMID (PMCID)
28511027
 FEMALE Infant
Ross-Konno Procedure With Cylinder Mitral Valve Replacement in 49 Days Old Infant.
Fedevych O, Yachnik O, Mykychak Y, Ivanov Y, Borodinova O, Yemets I.
World J Pediatr Congenit Heart Surg. 2018;9(5):587-590.
A neonate with antenatally diagnosed severe aortic valve stenosis, left ventricular outflow tract obstruction, left ventricular endocardial fibroelastosis, and severe mixed mitral valve disease was admitted to our center for balloon aortic valvuloplasty.
24826287
(4008507)
 OTHER
Heart Transplantation in a 14-Year-Old Boy in the Presence of Severe Out-of-Proportion Pulmonary Hypertension due to Restrictive Left Heart Disease: A Case Report.
Schwienbacher M, Schweigmann U, Neu N, Schermer E, Velik-Salchner C, Michel-Behnke I, Irnberger E, Steger CM, Stein JI, Geiger R.
Case Rep Cardiol. 2013;2013:418565.
A 14-year-old boy after balloon valvuloplasty of severe aortic valve stenosis in the neonatal period was referred for heart-lung transplantation because of high grade pulmonary hypertension and left heart dysfunction due to endocardial fibroelastosis with severe mitral insufficiency.
22922566
 MIXED_SAMPLE Infant, Newborn
Unusual aortic valve anomaly in the fetus: a case report.
Fesslova V, Boschetto C, Brankovic J, Bonacina E.
Fetal Diagn Ther. 2012;32(3):221-4.
Aortic valve anomalies in fetal life usually concern aortic valve stenosis, in severe forms associated to left ventricular impairment - endocardial fibroelastosis and mitral valve insufficiency.
6167022
 MALE Infant
Mitral insufficiency and aortic valve stenosis in infancy associated with endocardial fibroelastosis, surgical treatment and long-term results.
Di Benedetto G, Tiraboschi R, Ferrazzi P, Annecchino P, Gamba A, Parenzan L.
Thorac Cardiovasc Surg. 1981;29(3):192-4.
Mitral insufficiency and aortic valve stenosis in infancy associated with endocardial fibroelastosis, surgical treatment and long-term results.
6167022
 MALE Infant
Mitral insufficiency and aortic valve stenosis in infancy associated with endocardial fibroelastosis, surgical treatment and long-term results.
Di Benedetto G, Tiraboschi R, Ferrazzi P, Annecchino P, Gamba A, Parenzan L.
Thorac Cardiovasc Surg. 1981;29(3):192-4.
Surgical treatment of mitral insufficiency and aortic valve stenosis associated with endocardial fibroelastosis has not been well documented.