Congenital heart disease — when the heart, or blood vessels near the heart, don’t develop normally before birth — is one of the most common birth defects in the United States. It affects 1% of births per year.
At the Heart Institute at UPMC Children’s Hospital of Pittsburgh, our experts care for all types of congenital heart disease, including Ebstein’s anomaly.
Here, Luciana da Fonseca da Silva, MD, a cardiothoracic surgeon at the Da Silva Center for Ebstein’s Anomaly at UPMC Children’s, answers your questions about Ebstein’s anomaly.
What Is Ebstein’s Anomaly?
Ebstein’s anomaly is a rare, congenital heart defect of the tricuspid valve, which separates the right ventricle from the right atrium. This malformation causes blood to leak into the right atrium from the wrong direction, resulting in reduced blood flow.
While present at birth, Ebstein’s anomaly may not cause symptoms until childhood or even adulthood.
What are the symptoms of Ebstein’s anomaly?
Common Ebstein’s anomaly symptoms include:
- Abnormal heart rhythms (arrhythmias).
- Blue coloring of the lips and/or skin (cyanosis).
- Cough.
- Failure to grow (in the case of a child).
- Fatigue.
- Shortness of breath.
How Is Ebstein’s Anomaly Diagnosed?
Babies born with Ebstein’s anomaly may have low oxygen levels or a bluish tint to their skin. To diagnose children or adults, doctors may order the following tests:
- Electrocardiogram (EKG).
- MRI of the heart.
- Echocardiogram (echo).
- X-ray of the chest.
What Are the Treatment Options for Ebstein’s Anomaly?
Mild cases of Ebstein’s anomaly often have no symptoms and may not require treatment. Doctors can manage mild to moderate cases with symptoms with medications, such as:
- Anticoagulants to prevent blood clots, if the condition has caused heart complications.
- Diuretics to prevent water retention.
- Drugs to treat heart failure as a complication
For severe cases, the Da Silva Center offers surgical options, including the cone procedure and the Starnes procedure.
What is the cone procedure?
The cone procedure, pioneered by José Pedro da Silva, MD, is now the standard surgical treatment for people with Ebstein’s anomaly.
This technique uses a person’s tissue to reconstruct the tricuspid valve into a cone shape. Because the reconstructed valve is from their own tissue, the body is more willing to accept it. When a child has the cone procedure, the new valve continues to grow with them, reducing the need for additional intervention.
The expert team at the Da Silva Center can perform this procedure on people of all ages.
What is the Starnes procedure?
The Starnes procedure is a low-risk option to temporarily improve circulation in newborns with severe cases of Ebstein’s anomaly.
During the Starnes procedure, the surgeon closes the leaking tricuspid valve. They also create an extra connection between the right atrium and the right ventricle to provide blood flow to the lungs.
This procedure serves as a bridge until the child can withstand a more complex surgery, such as the cone procedure.
Why Should People Choose the Da Silva Center for Ebstein’s Anomaly Care?
The Da Silva Center is a leading destination for Ebstein’s anomaly care. We offer expert treatment for people of all ages, including those with complex valve abnormalities.
Our nearly 30 years of experience include performing more than 360 cone repairs. Our surgical team is highly skilled, ensuring exceptional outcomes.
Children benefit from a comprehensive Heart Center, while adults receive lifelong support, with a smooth transition from pediatric to adult services. The Da Silva Center’s commitment to excellence, innovation, and continuity of care makes it a top choice for those seeking care for Ebstein’s anomaly.
About the Da Silva Center for Ebstein’s Anomaly
At the Da Silva Center for Ebstein’s Anomaly, part of the Heart Institute at UPMC Children’s Hospital of Pittsburgh, our multidisciplinary team of specialists provides a world-class experience to people with Ebstein’s anomaly. Led by Luciana da Fonseca da Silva, MD, alongside José da Silva, MD, our team of doctors, nurses, and other health care professionals cares for people of all ages with this congenital heart defect.
You can contact the Da Silva Center at dasilvacenter@upmc.edu or 412-692-5218, or through our online form.