title: A mouse model of endocardial fibroelastosis.
abstract: BACKGROUND
  Endocardial fibroelastosis (EFE) is a pathologic condition of abnormal deposition of collagen and elastin within the endocardium of the heart. It is seen in conjunction with a variety of diseases including hypoplastic left heart syndrome and viral endocarditis. While an experimental model using heterotopic heart transplant in rats has been described, we sought to fully describe a mouse model that can be used to further elucidate the potential mechanisms of and treatments for EFE.
  
  
  METHODS
  The hearts of 2-day-old C57BL/6 mice were transplanted into the abdomen of 7-week-old C57BL/6 mice. At 2 weeks, the hearts were harvested and histologic analysis was performed using hematoxylin and eosin, Masson's trichrome, Russell-Movat's pentachrome, Picrosirius red, Hart's, Verhoeff-Van Gieson, and Weigert's Resorcin-Fuchsin stains. Additionally, one heart was analyzed using transmission electron microscopy (TEM).
  
  
  RESULTS
  Specimens demonstrated abnormal accumulation of both collagen and elastin within the endocardium with occasional expansion into the myocardium. Heterogeneity in extracellular matrix deposition was noted in the histologic specimens. In addition, TEM demonstrated the presence of excess collagen within the endocardium.
  
  
  CONCLUSIONS
  The heterotopic transplantation of an immature heart into a mouse results in changes consistent with EFE. This model is appropriate to investigate the etiology and treatment of EFE.
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA; Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, 1900 Coffey Road, Columbus, OH 43210, USA.
authors.forename: Elizabeth S
authors.initials: ES
authors.lastname: Clark
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA; Department of Pediatric Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205-2664, USA.
authors.forename: Victoria K
authors.initials: VK
authors.lastname: Pepper
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA.
authors.forename: Cameron A
authors.initials: CA
authors.lastname: Best
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA; Department of Surgery, The Ohio State University, 395 W. 12th Avenue - Suite 670, Columbus, OH 43210, USA.
authors.forename: Ekene A
authors.initials: EA
authors.lastname: Onwuka
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA.
authors.forename: Tai
authors.initials: T
authors.lastname: Yi
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA.
authors.forename: Shuhei
authors.initials: S
authors.lastname: Tara
authors.affiliation: Department of Veterinary Biosciences, College of Veterinary Medicine, The Ohio State University, 1900 Coffey Road, Columbus, OH 43210, USA.
authors.forename: Rachel
authors.initials: R
authors.lastname: Cianciolo
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA; Department of Pathology, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205-2664, USA.
authors.forename: Peter
authors.initials: P
authors.lastname: Baker
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA; Department of Cardiovascular Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205-2664, USA.
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Shinoka
authors.affiliation: Tissue Engineering Program, The Research Institute at Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA; Department of Pediatric Surgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH 43205-2664, USA. Electronic address: christopher.breuer@nationwidechildrens.org.
authors.forename: Christopher K
authors.initials: CK
authors.lastname: Breuer
mesh_terms: D000367:Age Factors; D000818:Animals; D003094:Collagen / Q000378:metabolism; D004195:Disease Models, Animal; D004549:Elastin / Q000378:metabolism; D004695:Endocardial Fibroelastosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D004699:Endocardium / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D005109:Extracellular Matrix / Q000378:metabolism; D016027:Heart Transplantation; D008810:Mice, Inbred C57BL; D013997:Time Factors; D014463:Ultrasonography
chemical_list: D003094:Collagen; D004549:Elastin
keywords: Congenital heart disease; EFE; Endocardial fibroelastosis; Mouse model; Unloaded heterotopic heart transplant