RT Book, Section A1 Groner, Jonathan I. A2 Ziegler, Moritz M. A2 Azizkhan, Richard G. A2 Allmen, Daniel von A2 Weber, Thomas R. SR Print(0) ID 1100432665 T1 Ectopia Cordis and Sternal Defects T2 Operative Pediatric Surgery, 2e YR 2014 FD 2014 PB McGraw-Hill Education PP New York, NY SN 978-0-07-162723-8 LK accesspediatrics.mhmedical.com/content.aspx?aid=1100432665 RD 2024/03/28 AB The nomenclature for ectopia cordis is confusing and inaccurate; only a few survivors of “true” ectopia cordis have ever been reported.Thoracic ectopia cordis or “true” ectopia cordis is an extremely rare condition in which the bare heart beats outside the body on the newborn's chest, and is frequently tilted so that the apex is near the patient's chin.Thoracoabdominal ectopia cordis is an omphalocele variant involving major deficiencies of the abdominal wall (superior to and including the umbilicus), diaphragm, and lower sternum. Although the heart may protrude (and may be visible), it is typically covered with attenuated pericardium or skin, and is properly oriented.Sternal cleft is a condition in which there is a deficit in the superior sternum. In some patients with large clefts, the heart appears to protrude out of the chest when the patient performs a Valsalva maneuver.Patients with ectopia cordis often have structural abnormalities of the heart, whereas patients with sternal clefts usually have normal hearts.Sternal clefts can usually be repaired in a single stage with primary closure or autologous tissues. In contrast, repairs of ectopia cordis generally require staged operations and the use of prosthetic materials.