title: Ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia.
abstract: Ectopia cordis, defined as partial or complete displacement of the heart outside of the thoracic cavity, is a rare congenital malformation. If not surgically corrected during the early years of life, ectopia cordis can prove to be a fatal abnormality. However, due to the presence of multiple intracardiac and extracardiac malformations, a corrective surgery might not always be successful. The pathology of ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia is discussed, highlighting the complexities involved in such a rare disorder.
authors.affiliation: Cardiovascular Center, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts.
authors.forename: Rabiya
authors.initials: R
authors.lastname: Malik
authors.forename: Mark V
authors.initials: MV
authors.lastname: Zilberman
authors.forename: Liwen
authors.initials: L
authors.lastname: Tang
authors.forename: Susan
authors.initials: S
authors.lastname: Miller
authors.forename: Natesa G
authors.initials: NG
authors.lastname: Pandian
mesh_terms: D000328:Adult; D004452:Echocardiography / Q000379:methods; D054083:Ectopia Cordis / Q000000981:diagnostic imaging*; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000000981:diagnostic imaging*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D006801:Humans; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000000981:diagnostic imaging*; D035583:Rare Diseases / Q000000981:diagnostic imaging; D014188:Transposition of Great Vessels / Q000150:complications / Q000000981:diagnostic imaging*
keywords: double outlet right ventricle; ectopia cordis; left ventricular hypoplasia; malposed great arteries; pentalogy of Cantrell; ventricular septal defect