Ectopia cordis

Ectopia cordis is a birth defect in which the heart is abnormally located. In the most common form, the heart protrudes outside the chest through a split sternum. Less often the heart may be situated in the abdominal cavity or neck.

Often other birth defects are also present. This condition is usually fatal in the first days of life. In some cases surgical treatment is possible. The ectopic heart is not protected by the skin or sternum.

Other organs may also have formed outside the skin. Often the heart is not formed properly and many other heart defects are associated with this condition including: Tetralogy of Fallot, pulmonary atresia, atrial and ventricular septal defects, double outlet right ventricle.

Other non cardiac malformations may be present such as cleft palate, spine malformations that can cause kyphosis.

Occurrence
7.9 per million births. Ectopia cordis is a very rare congenital heart malformation.

Etiology
No exact cause has been identified but this condition has also been seen more frequently in Turner Syndrome and Trisomy 18; however so far there is no evidence that it is a genetically transmissible disease.

Prognosis
Most cases result in stillbirth or death shortly after birth. Some cases of ectopia cordis can be treated surgically but in general involve lengthy and very complicated pediatric cardiothoracic surgery.

Depending on the position of the heart from birth ectopia cordis can be classified into four different categories:


 * Cervical
 * Thoracic: where the heart would lie within the thoracic cavity.
 * Thoracoabdominal: where the heart would lie somewhere between the thoracic and abdominal cavities.
 * Abdominal: where the heart would lie in the abdominal cavity.

The malfunction happens when the sternum forms during the gestation period and the heart begins to grow in the wrong place.