WHEN THE HEART FORMS OUTSIDE THE BODY.

WHAT IS ETOPIC CORDIS? Ectopia cordis is an extremely rare congenital heart malformation that remains very difficult to treat. Occurrence is .79 in every 10,000 births. In ectopia cordis, the heart forms outside of the chest wall, so it is not protected by the skin or sternum. Other organs may also have formed outside the skin. To further complicate this difficult presentation, the heart itself is generally improperly formed. It may have defects like Tetralogy of Fallot, pulmonary atresia, atrial and ventricular septal defects, or double outlet right ventricle, among others. Along with heart malformations, children with ectopia cordis may also have cleft palates. The spine can also be formed improperly, causing kyphosis, too great of a C curvature in the thoracic spine. There is some slim evidence that ectopia cordis may be related to Turner Syndrome. Chromosomal anomalies like Trisomy 18 have also been observed in some children with ectopia cordis. In general, however, there is no recognizable cause for this condition. In one way, this may be comforting to parents who have had a child with ectopia cordis. The condition is unlikely to repeat in future children. Sadly, most cases of ectopia cordis result in stillbirth or death shortly after birth. A few cases of ectopia cordis have been treated successfully, but this defect still poses the biggest challenge and rate of failure for pediatric cardiothoracic surgeons.