RT Book, Section A1 Bianchi, Diana W. A1 Crombleholme, Timothy M. A1 D'Alton, Mary E. A1 Malone, Fergal D. SR Print(0) ID 1106398608 T1 Pentalogy of Cantrell T2 Fetology: Diagnosis and Management of the Fetal Patient, 2e YR 2015 FD 2015 PB McGraw-Hill Education PP New York, NY SN 978-0-07-144201-5 LK obgyn.mhmedical.com/content.aspx?aid=1106398608 RD 2024/03/28 AB Key PointsPentalogy of Cantrell is defined by the association of five anomalies: (1) epigastric abdominal wall defect, (2) lower sternal defect, (3) defect in the anterior diaphragm, (4) defect in the epicardium, and (5) intracardiac defects.Can be associated with other midline ventral defects such as cleft lip and palate, exencephaly, and sirenomelia.Rare association thought to occur in only 1 in 65,000 to 200,000 births.Most common intracardiac defects include VSD, ASD, pulmonic stenosis, tetralogy of Fallot, and LV diverticulum.All the defects are amendable to repair yet outcomes in this condition have been generally poor.An X-linked pattern of inheritance has been suggested for ventral midline defects including pentalogy of Cantrell.