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What Is Ebstein’s Anomaly?

Ebstein’s anomaly is a very rare congenital heart defect. Babies with Ebstein's anomaly have a tricuspid valve that did not form correctly. Normally the tricuspid valve has three flaps or leaflets. It works like a one-way door in the heart that allows blood to flow from the right atrium (one of the atria of the heart) to the right ventricle.

In Ebstein's anomaly, one or two of the flaps are malformed and do not move normally. The valve does not work well, and blood may leak backwards from the right ventricle into the right atrium with each heartbeat. This may cause the right atrium to enlarge.

This condition is also called Ebstein’s malformation of the tricuspid valve. Doctors do not know what causes this heart malformation.

Ebstein’s Anomaly in Children

Children with Ebstein’s anomaly often have a hole in the septum between their atria, called an atrial septal defect. This allows oxygen-poor (blue) blood to be pumped out to the body and can sometimes cause cyanosis.

Some children with this heart defect also have Wolff-Parkinson-White syndrome. This along with an enlarged right atrium may increase the risk that children with Ebstein’s anomaly will have a heart rhythm disorder.

Ebstein’s Anomaly at Seattle Children’s

Our heart team has treated many children with Ebstein’s anomaly. We have extensive experience with the treatment these patients may require, including medicines or surgery. We also have a pediatric cardiac anesthesia team and a cardiac intensive care unit ready to care for children who undergo heart surgery.

When you come to Children's, a team of people will take care of your child. Along with your child's cardiologist, you are connected with neonatologists, pulmonologists (lung doctors), nurses, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child's health needs and help your family through this experience.

Since 1907, Children's has been treating children only. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. Our expertise in pediatrics truly makes a difference for our patients and families.

The Adult Congenital Heart Disease Program shared by Children’s and the University of Washington can help with care throughout your child’s life.

Who Treats This at Seattle Children's?

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Summer 2014: Good Growing Newsletter

In This Issue

  • Understanding the Power and Influence of Role Models
  • Legal Marijuana Means Greater Poisoning Risks for Children
  • Why Choose Pediatric Emergency Care?

Download Summer 2014 (PDF)