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.