Xingbo Xu 1 , Ingeborg Friehs 1 , Tachi Zhong Hu 1 , Ivan Melnychenko 1 , Björn Tampe 1 , Fouzi Alnour 1 , Maria Iascone 1 , Raghu Kalluri 1 , Michael Zeisberg 1 , Pedro J Del Nido 1 , Elisabeth M Zeisberg 2
Feb 27 2015
Endocardial fibroelastosis (EFE) is a unique form of fibrosis, which forms a de novo subendocardial tissue layer encapsulating the myocardium and stunting its growth, and which is typically associated with congenital heart diseases of heterogeneous origin, such as hypoplastic left heart syndrome. Relevance of EFE was only recently highlighted through the establishment of staged biventricular repair surgery in infant patients with hypoplastic left heart syndrome, where surgical removal of EFE tissue has resulted in improvement in the restrictive physiology leading to the growth of the left ventricle in parallel with somatic growth. However, pathomechanisms underlying EFE formation are still scarce, and specific therapeutic targets are not yet known.