Ebstein's Anomaly

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with Ebstein’s anomaly, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

 
What Is It?

Ebstein's anomaly is a rare defect, accounting for less than 1 percent of all congenital heart defects. It consists of abnormal development of the tricuspid valve, which is located between the right atrium (one of the upper chambers of the heart) and the right ventricle (one of the heart’s two lower chambers). Blood passes through the tricuspid valve after returning from the body and before being pumped to the lungs.

Ebstein’s Results from Abnormal Development of the Tricuspid Valve

Two of the three leaflets of the valve (the “septal leaflet” and the “posterior leaflet”) don’t form normally. Rather than being located where the right atrium and ventricle meet, they’re displaced downward into the right ventricular cavity. These valve leaflets vary from mildly deformed to severely deformed. The third leaflet (called the “anterior leaflet”) is in the correct position but is typically large and floppy — it’s often described as “sail-like.” The portion of the right ventricle that sits above the leaflets is usually thinner than normal and may be referred to as the “atrialized portion” of the right ventricle.

Other Defects Also Present in Ebstein’s Anomaly

A hole between the upper chambers of the heart (an atrial septal defect or ASD) is almost always present in association with Ebstein’s anomaly. In some children with this malformation, the pulmonary valve (the heart valve between the right ventricle and the pulmonary artery that consists of three flaps, or “cusps”) is also abnormal, either abnormally tight (called “pulmonary valve stenosis”) or entirely closed (called “pulmonary valve atresia”).

How Ebstein’s Anomaly Affects Blood Flow Through The Heart

Most commonly, the deformed tricuspid valve leaks — and it may leak severely. That means that as the right ventricle contracts, some blood flows backward from the right ventricle to the right atrium, rather than flowing forward toward the lungs. The right atrium, which receives this leaking blood, is usually enlarged, as is the right ventricle. In severe cases, the right ventricle may be so large that it pushes on the left ventricle and prevents the left ventricle from filling normally.

Often, some of the oxygen-poor (“blue”) blood from the right atrium passes across the hole in the atrial septum (the wall between the right and left atria — the top two chambers of the heart) into the left atrium. This blue blood then goes out to the body, which may cause cyanosis (bluish color in the skin due to insufficient oxygen). Because the resistance to blood flow through the lungs (this is called “pulmonary vascular resistance”) decreases in the days and weeks after birth, the cyanosis may improve in mild cases of Ebstein’s anomaly.

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:

 
How & When Is It Diagnosed?

In severe cases, Ebstein’s anomaly may be diagnosed on a fetal echocardiogram (“echo”) during pregnancy, or it may be delayed until after birth. When babies are born with Ebstein’s anomaly, the timing of when children will show symptoms will depend on a number of factors:

  • the anatomy of the pulmonary valve
  • the degree of leakage of the tricuspid valve
  • whether or not the right ventricular size is interfering with the normal function of the left ventricle

In very mild cases, diagnosis is usually the result of hearing a murmur and — in rare cases — may not occur until the child is older. In cases with significant pulmonary stenosis or atresia, oxygen levels in the blood may be low. An echocardiogram (“echo”) is usually enough to make the diagnosis, and children rarely need additional testing. An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.

 
How & When Is It Treated?

Children who have a mild degree of valve deformity and no symptoms (they’re “asymptomatic”) may not need any treatment. However, children with more severe Ebstein's anomaly may have:

  • cyanosis (bluish color of the skin, which may be due to not enough oxygen)
  • shortness of breath
  • exercise intolerance (difficulty exercising)

Children with these symptoms or significant heart enlargement will likely need surgery. Depending on the child’s age and the severity of the anomaly, the operation may involve repairing or — rarely — replacing the abnormal tricuspid valve and closing the hole between the atria. Or, in some cases, where the enlargement of the right ventricle prevents that ventricular from working properly, children may need a series of operations called “single ventricle palliation”.

When newborns need surgery for Ebstein’s anomaly in the first month after birth, the risks are extremely high, partly due to underdevelopment of the lungs while in the womb.


Learn More
 
How Will It Affect My Child Long Term?

In more mild forms of Ebstein’s anomaly, children may never need an operation and often grow up normally.

Children with severe forms will likely need multiple operations over their lifetime. Among those who do need surgery early on, the enlargement of the atrium may cause problems with the heart’s electrical system and they may need a permanent pacemaker to prevent abnormal electrical impulses or “arrhythmias.” (Connected to the heart by a wire, a pacemaker is a small, battery-operated device implanted into the body, near the collarbone or at the bottom of the ribcage, through a surgical procedure. Through a sensing device, a pacemaker can detect if the heart rate is too slow and sends electrical signals to the heart to speed up the heartbeat.)

If your child has Ebstein’s anomaly, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

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Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


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Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »