Ectopia cordis

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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 present such as cleft palates, spine malformations that can cause kyphosis.

Occurrence: 7.9 per million births. Ectopia cordis is 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.