Ectopia cordis

Ectopia Cordis is a rare congenital condition characterized by the abnormal placement of the heart outside the thoracic cavity. This condition is usually associated with other congenital malformations, particularly those involving the diaphragm, abdominal wall, and pericardium.

Etiology

The exact cause of ectopia cordis is unknown. However, it is believed to result from a failure in the development of the ventral mesoderm and the anterior body wall. This failure leads to the abnormal placement of the heart. The condition is often associated with other congenital anomalies, such as omphalocele, gastroschisis, and pectus excavatum.

Epidemiology

Ectopia cordis is an extremely rare condition, with an estimated incidence of 5.5 to 7.9 per million live births. The condition is more common in males than in females.

Clinical Presentation

The clinical presentation of ectopia cordis can vary greatly depending on the location of the heart. In most cases, the heart is located outside the chest, either in the midline of the thorax or in the abdominal region. In some cases, the heart may be located in the neck or in the cranial region. The condition is often associated with severe cardiovascular compromise, leading to high mortality rates.

Diagnosis

The diagnosis of ectopia cordis is usually made prenatally through ultrasound imaging. The condition can be clearly seen as the heart is located outside the normal thoracic location. Postnatal diagnosis is made through physical examination and imaging studies.

Treatment

The treatment of ectopia cordis is surgical and aims to place the heart in the thoracic cavity and repair the associated defects. The prognosis is generally poor due to the associated anomalies and the complexity of the surgical procedure.

See Also

• Congenital heart defect
• Pentalogy of Cantrell
• Thoracic surgery