Endocardial fibroelastosis in infants and young children: a state-of-the-art review

Heart Fail Rev. 2023 Sep;28(5):1023-1031. doi: 10.1007/s10741-023-10319-0. Epub 2023 May 24.


Endocardial fibroelastosis (EFE) is a rare cardiac condition characterized by excessive endocardial thickening secondary to fibroelastic tissues that commonly present in infants and young children. Most of endocardial fibroelastosis cases are secondary forms, which occur in conjunction with other cardiac diseases. Endocardial fibroelastosis has been associated with poor prognosis and outcomes. In light of recent advancements in understanding pathophysiology, several new data have revealed compelling evidence that abnormal endothelial-to-mesenchymal transition is the root cause of endocardial fibroelastosis. This article aims to review the recent development in pathophysiology, diagnostic workup, and management, and to discuss possible differential diagnoses.

Keywords: Cell receptors; Cell signaling; Congenital heart disease; Endocardial fibroelastosis; Heart failure.

Publication types

  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Endocardial Fibroelastosis* / complications
  • Endocardial Fibroelastosis* / diagnosis
  • Endocardium
  • Humans
  • Infant