Ebstein Anomaly

Ebstein anomaly is a rare, complex congenital heart condition in which the anatomy (structure) of your child’s heart does not form correctly in the womb. It accounts for less than 1% of congenital heart disease in children.

Our approach to treating Ebstein anomaly

At Stanford Medicine Children’s Health, we specialize in caring for children with rare and highly complex heart conditions. Parents across the nation and world name the following reasons for coming to us for highly advanced, expert complex heart care, including for Ebstein anomaly:

  • We provide a complete evaluation from a large team of heart specialists. Ebstein anomaly is known among cardiologists as a highly variable condition that manifests uniquely in every child. Because our Betty Irene Moore Children’s Heart Center has heart specialists for every type of heart condition, we’re skilled at navigating the most complex situations, even when a baby’s heart health changes course quickly. To provide the best possible care, including a comprehensive evaluation, for your child with Ebstein anomaly, we bring together pediatric experts in echocardiography (heart ultrasound), advanced cardiac imaging, fetal cardiology, electrophysiology, cardiac catheterization, pulmonary hypertension, cardiovascular surgery and cardiovascular critical care. We consider your child’s heart needs from every angle, then develop a personalized treatment plan specific to them.
  • Our heart surgeons are known worldwide for their excellent heart surgery outcomes. If your child needs heart surgery now or in the future, our heart surgeons reliably succeed at procedures considered radical and daunting elsewhere, and are especially known for performing challenging surgeries on children with complex structural heart defects, including premature or low-birth-weight newborns. Our heart surgeons perform significantly more heart surgeries than the national average, which means more experienced and better outcomes for your child.
  • Our advanced care keeps your child’s heart working at its best. We support your child’s heart in functioning at its best, despite having Ebstein anomaly. Our effective, innovative treatments help your child live a healthy life with minimal symptoms and less need for future heart surgeries. If your child is seeing a community provider, we are happy to partner with your child’s referring cardiologist to provide seamless care.

About Ebstein anomaly

The following facts will help you understand Ebstein anomaly:

  • In Ebstein anomaly, the tricuspid valve—the valve that manages blood flow from the upper to lower right chambers of the heart—is misshapen or in the wrong position.
  • When the tricuspid valve doesn’t function correctly, the heart has to work harder to push blood forward, especially when blood is flowing backward through the leaky valve. This can strain the heart.
  • An echocardiogram can identify Ebstein anomaly before your child is born. A baby’s physiology is very different before and after birth, so doctors often must wait until birth to see the effects of Ebstein anomaly. Rest assured, we will carefully weigh and evaluate all possible health scenarios for your child upon birth.
  • Ebstein anomaly can be diagnosed at any age, but the sooner it is diagnosed, the better. When our obstetric/maternal-fetal medicine team recognizes that your developing baby’s heart does not appear normal on ultrasound, you are referred to our fetal cardiology team.

Signs of Ebstein anomaly

  • Bluish tint to the skin and lips (cyanosis).
  • Heart murmur.
  • Arrhythmias, usually very fast heart rates.
  • Poor growth.
  • Dizzy spells.
  • Shortness of breath.
  • Unable to exercise like their peers.

Treatments for Ebstein anomaly

Ebstein anomaly ranges from mild to severe, and treatment varies depending on how well your child’s tricuspid valve is working. We provide just the right care at just the right time to optimize your child’s health now and in the future. Below is the general approach we take to treating Ebstein anomaly; however, we personalize care for every child.

  • When Ebstein anomaly is mild, your child may not need surgery and may simply need to be observed or receive medication to help their heart work better.
  • With moderate structural problems in the tricuspid valve, surgery may be needed as your baby or child grows.
  • When Ebstein anomaly is severe, there is a good chance that surgery is needed right after birth or shortly after—especially if your child is cyanotic (bluish skin), a sign of low oxygen.
  • In the most severe cases, a single ventricle approach might be required.
  • It is not uncommon for children with Ebstein anomaly to have heart rhythm problems (arrhythmia). We test all children diagnosed with Ebstein anomaly for possible heart rhythm issues.

Learn more about our exceptional heart care for children with simple to complex conditions >