Endocardial fibroelastosis is caused by aberrant endothelial to mesenchymal transition.

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Abstract

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.

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Journal

Journal ID (iso-abbrev): Circ. Res.

Title: Circulation research

ISSN (Electronic): 1524-4571

ISSN (Print): 0009-7330

Publication date (Electronic): Feb 27 2015

Volume: 116

Issue: 5

Affiliations

[1 ] From the Department of Cardiology and Pneumology (X.X., F.A., E.M.Z.), Department of Nephrology and Rheumatology (B.T., M.Z.), University Medical Center of Göttingen, Georg-August University, Göttingen, Germany; Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, MA (I.F., I.V., P.J.d N.); Division of Matrix Biology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (T.Z.H., R.K., E.M.Z.); Lab Genetica Molecolare, Papa Giovanni XXIII Hospital, Bergamo, Italy (M.I.); Department of Cancer Biology and the Metastasis Research Center, University of Texas MD Anderson Cancer Center, Houston (R.K.); and DZHK (German Centre for Cardiovascular Research), partner site Göttingen, Germany (E.M.Z.).

[2 ] From the Department of Cardiology and Pneumology (X.X., F.A., E.M.Z.), Department of Nephrology and Rheumatology (B.T., M.Z.), University Medical Center of Göttingen, Georg-August University, Göttingen, Germany; Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, MA (I.F., I.V., P.J.d N.); Division of Matrix Biology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (T.Z.H., R.K., E.M.Z.); Lab Genetica Molecolare, Papa Giovanni XXIII Hospital, Bergamo, Italy (M.I.); Department of Cancer Biology and the Metastasis Research Center, University of Texas MD Anderson Cancer Center, Houston (R.K.); and DZHK (German Centre for Cardiovascular Research), partner site Göttingen, Germany (E.M.Z.). Elisabeth.zeisberg@med.uni-goettingen.de.

Article

Publisher Item ID: CIRCRESAHA.116.305629 Mid ID: NIHMS656707

DOI: 10.1161/CIRCRESAHA.116.305629

PMC ID: 4344885

PubMed ID: 25587097

SO-VID: 75b4bc1e-cb33-4d4f-82e5-c8a0043b5207

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Keywords: congenital heart disease,endocardial fibroelastosis,endocardium,endothelial cell,fibrosis,hypoplastic left heart syndrome

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Keywords: congenital heart disease, endocardial fibroelastosis, endocardium, endothelial cell, fibrosis, hypoplastic left heart syndrome

Endocardial fibroelastosis is caused by aberrant endothelial to mesenchymal transition.

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