title: Pentalogy of Cantrell with ectopia cordis totalis, total anomalous pulmonary venous connection, and tetralogy of Fallot: a case report and review of the literature.
abstract: Pentalogy of Cantrell is a rare condition with a varied expression and a high mortality. We present a patient with the classic pentad (type 1), but with a previously undescribed constellation of cardiac manifestations including ectopia cordis totalis, total anomalous pulmonary venous return, and tetralogy of Fallot. This case reminds us of the challenges associated with the management of various forms of this condition. We discuss the prenatal diagnosis, genetic basis, postnatal evaluation, and management of this entity.
authors.affiliation: Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children's Hospital-Carver College of Medicine, Iowa City, Iowa, USA.
authors.forename: M Santiago
authors.initials: MS
authors.lastname: Restrepo
authors.forename: Amanda
authors.initials: A
authors.lastname: Cerqua
authors.forename: Joseph W
authors.initials: JW
authors.lastname: Turek
mesh_terms: D000015:Abnormalities, Multiple; D004452:Echocardiography; D054083:Ectopia Cordis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D058502:Pentalogy of Cantrell / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D010690:Phlebography / Q000379:methods; D011247:Pregnancy; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D013771:Tetralogy of Fallot / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D014057:Tomography, X-Ray Computed; D016216:Ultrasonography, Prenatal / Q000379:methods; D055815:Young Adult
keywords: Ectopia Cordis; Pentalogy of Cantrell; Tetralogy of Fallot