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Endocardial Fibroelastosis
Classification and external resources
ICD-9 425.3
OMIM 226000 305300

Endocardial Fibroelastosis (EFE) is a rare heart disorder usually associated with children two years old and younger.[1]

It should not be confused with endomyocardial fibrosis.

Contents

Presentation

It is characterized by a thickening within the muscular lining of the heart chambers (the endocardium) due to an increase in the amount of supporting connective tissue and elastic fibers.

It is an uncommon cause of unexplained heart failure in infants and children.

It is one component of HEC syndrome.

Causes

EFE has two distinct genetic forms, each having a different mode of inheritance. An x-linked recessive form,[2] and an autosomal recessive form[3] have both been observed.

There is also some evidence that viral infection may play a role as a cause or trigger.[4]

History

The term was introduced by Weinberg and Himmelfarb in 1943.[5]

Treatment

One form of treatment is heart transplant.[6]

See also

References

  1. ^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. pp. 607. ISBN 0-7216-0187-1.  
  2. ^ Online 'Mendelian Inheritance in Man' (OMIM) 226000
  3. ^ Online 'Mendelian Inheritance in Man' (OMIM) 305300
  4. ^ Ni J, Bowles NE, Kim YH, et al. (January 1997). "Viral infection of the myocardium in endocardial fibroelastosis. Molecular evidence for the role of mumps virus as an etiologic agent". Circulation 95 (1): 133–9. PMID 8994428. http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=8994428.  
  5. ^ Weinberg T, Himmelfarb AJ (1943). "Endocardial fibroelastosis". Bull Johns Hopkins Hosp. 72: 299.
  6. ^ Netz H, Bauer J J, Scheld HH, et al. (1990). "Cardiac transplantation in a neonate with endocardial fibroelastosis". Tex Heart Inst J 17 (2): 122–5. PMID 15227396.  
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