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What is ectopia cordis?

Ectopia cordis is a rare congenital condition in which some or all of a baby’s heart doesn’t have the typical coverage of the breastbone. The heart instead lies beneath a layer of skin and appears to be outside the chest.

The newborn heart has little protection from injury when it is in this position, so it’s imperative to move it into proper place as soon as the infant is healthy and strong enough for surgery. The term “ectopia cordis” is Latin: “ectopia” means “outside” and “cordis” means “heart.”

Symptoms & Causes

What are the symptoms of ectopia cordis?

Ectopia cordis is evident at birth. In most cases, the newborn’s heart appears to sit outside the chest. In other instances, it has developed in line with the stomach or is between the chest and stomach. In rare cases, the heart sits higher, near the neck. The heart can be partially protected by the thoracic cavity, but if it is outside the cavity, it is usually covered by skin or serous membrane and not properly protected from harm.

What causes ectopia cordis?

Ectopia cordis is caused by an abnormal formation of the chest wall and abdominal wall structure. Babies with this condition can also have other congenital malformations of the heart — including double outlet right ventricle (DORV), a condition in which the heart’s two major arteries both connect to the right ventricle. Ectopia cordis is associated with stomach malformations such as omphalocele, when abdominal organs protrude through an opening in muscles around the umbilical cord.

Diagnosis & Treatments

How is ectopia cordis diagnosed?

Ectopia cordis can be detected before birth through ultrasound scans. It is found in one baby per 5.5 million births. It tends to occur more in males.

How is ectopia cordis treated?

Ectopia cordis is a life-threatening condition. The Complex Biventricular Repair Program at the Benderson Family Heart Center at Boston Children’s Hospital works quickly to stabilize the health and strength of babies before surgically placing the heart inside the chest and closing the thoracic cavity. A child might also require other operations, including the repair of other malformations of the heart and stomach, or the building of a sternum to protect the heart.

How we care for ectopia cordis

Our team works closely with surgeons and clinicians from other medical disciplines to treat this complex malformation. We also provide support to parents and family, discussing every detail of the surgery and treatment plan, and explaining what they can expect throughout the process.