Ectopia Cordis is a rare condition in which the heart is located partially or completely outside of the thoracic cavity. Often, it is associated with pentalogy of Carntrell, which is a rare thoraco-abdominal disruption or a defect in the abdominal wall. This condition is categorized into five types: cervical, abdominal, thoracocervical, thoracoabdominal, and thoracic. Ectopia cordis is extremely uncommon as it only occurs in 1:126,000 births. The prognosis for this condition is poor and depends on the severity of the condition. The infants affected by ectopia cordis are usually stillborn. Surgical correction is often attempted, although infants are often stillborn or die within days after birth.
Ectopia cordis is defined as a rare congenital disorder in which the heart is located either partially or completely outside of the thoracic cavity. Ectopia cordis is often associated with pentalogy of Cantrell, a rare disorder in which there is a thoracoabdominal disruption or defect in the abdominal wall.
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The prognosis of ectopia cordis is typically poor as the majority of infants die within the first few days of birth or are stillborn. Although if recommended, surgical correction can be attempted immediately to cover the exposed heart and abdominal contents. Although this typically results in intolerable hemodynamic embarrassment, caused by damage to the great vessels or compression of the heart. Surgical correction involves the use of silastic prosthesis, sternal or thoracic reconstruction, or placement of the heart into the thoracic cavity. Although, before closing the abdominal wall, there should be a complete correction of the intracardiac defects. There have been two reported survivors out of twenty-nine