Children's Hospital Colorado

Ectopia Cordis

What is ectopia cordis?

Ectopia cordis occurs when a portion or all of a baby's heart is located outside of the chest cavity. In true ectopia cordis, the heart almost always has internal abnormalities aside from just the abnormal location. At the Colorado Fetal Care Center at Children's Hospital Colorado, we work with expectant mothers and families to diagnose this severe cardiac defect.

Babies with ectopia cordis are born with their hearts partially or completely outside of their bodies. There are usually other organ structures that also develop abnormally. Ectopia cordis often coexists with additional heart defects as well as abdominal wall defects.

Types of ectopia cordis

There are four ectopia cordis classifications or "types" based on the position of the heart:

  • Cervical (3% of cases)
  • Thoracic (64% of cases)
  • Thoracoabdominal ectopia (18% of cases)
  • Abdominal ectopia (15%)

The type of ectopia cordis your child has can also depend on whether the heart is totally uncovered, covered by a serous membrane or covered by skin. Depending on the type of ectopia cordis, as well as the associated heart defects, our team will be better able to predict the outcomes and treatment options for your child after birth.

What causes ectopia cordis?

Ectopia cordis is extremely rare, affecting around one in 126,000 births. The cause of ectopia cordis is unknown, but males tend to be affected more often than females. There has not been a reported case of recurrence of ectopia cordis in a sibling.

What are the expected outcomes for a baby with ectopia cordis?

Unfortunately, the ectopia cordis survival rate is just 10%. Most babies born with hearts outside their bodies have severe intracardiac abnormalities are stillborn or die within the first few days of life. Those who survive require extensive surgeries and lifelong medical care delivered by a team of specialists.

For parents, receiving news that your baby has such a serious condition can be devastating. The Colorado Fetal Care Center is a national leader in diagnosing and treating ectopia cordis, and we are here to provide families with support throughout their time with us.

How is ectopia cordis diagnosed?

This condition is often diagnosed by ultrasound and can occur as early as the first trimester. If not discovered during pregnancy, it becomes obvious as soon as the baby is born.

What are the treatment options for ectopic cordis?

Ectopia cordis treatment varies based on your child's specific needs. At the Colorado Fetal Care Center, we will consider your child's condition before choosing the treatment options that will provide the best possible outcomes.

In utero options

When a baby is diagnosed with this severe birth defect in utero, the mother can opt to terminate the pregnancy, as the ectopia cordis survival rate is around 10%. There are currently no fetal interventions (surgery) options while the baby is in utero.

Post-birth surgery

Ectopia cordis treatment begins with emergency surgery after birth to place the heart inside the baby's chest and close the thoracic cavity. Additional operations entail building a sternum to protect the heart and repairing any additional heart or abdominal wall defects.

Throughout diagnosis and treatment, Children's Colorado provides families with support groups and other helpful resources in addition to cutting-edge medical care.

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