Treatment & Care
Having identified your child’s heart condition, at Children’s Hospital Boston we’re able to begin the process of treating him, so that we may ultimately return him to good health.
Medication for milder cases. Most children who have a milder form of Ebstein’s anomaly can be managed with medication to control:
- the fluid build-up that causes congestive heart failure
- abnormal heart rhythms (arrhythmias)
- abnormal heart rate
Surgery for more severe cases. But if your child’s condition is severe—causing low levels of oxygen in the blood (cyanosis) or arrhythmia—his situation will probably be urgent, and surgery will be needed.
Your baby will most likely be admitted to Children’s cardiac intensive care unit (CICU) once his symptoms become apparent. Initially, he may be placed on oxygen or a ventilator to help him breathe, and IV (intravenous) medications may be given to help his heart and lungs function more efficiently.
There are several surgical approaches to repairing your child’s tricuspid valve, closing his patent foramen ovale (PFO), and repairing other defects (such as ASD) that are associated with Ebstein’s anomaly. Surgical techniques include:
surgical valve repair: surgeons will reposition the existing valve, reconfigure the shape of the tricuspid valve leaflets, and suture an annular ring into place.
cone procedure: Children’s is one of just a handful of heart centers in the United States that performs the advanced and innovative cone procedure for repairing the tricuspid valve in children with Ebstein’s anomaly.
In this procedure, extra tissues on the enlarged right side of the heart are folded up, and the malformed valve is surgically reshaped into a cone. The cone-shaped valve opens to a central blood flow and closes with full coaptation (joining, reuniting) of the leaflets. (See the Patient story in this topic.)
- tricuspid valve replacement: Sometimes a patient’s tricuspid valve is too malformed to be repaired. In this case, the existing valve will need to be replaced with a mechanical valve or a bioprosthetic one made of human or animal tissue.
Radiofrequency catheter ablation treatment for arrhythmias. For children whose condition includes significant arrhythmias and heart beat irregularities that can’t be fully controlled by medication, doctors can perform a catheterization intervention called radiofrequency catheter ablation to treat these heart arrhythmias.
In 1990, Children’s doctors performed the first pediatric non-surgical repair of a cardiac arrhythmia, using catheter ablation to correct a rhythm disorder called Wolff-Parkinson-White syndrome. So, today, Children’s experts can call on our more than three decades’ worth of experience with this procedure.
At home: caring for your child after surgery
After surgery, your child’s cardiologist will offer recommendations for follow-up care, including:
- wound care while the baby is healing
- a nutritional program to encourage weight gain
- an oral hygiene program to prevent infection
- an appropriate exercise regimen to build body mass and achieve fitness
As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. Throughout his childhood, you can help your child—and the whole family—by making sure heeats a heart-healthy diet, exercises regularly, and as he grows into the teen years avoids smoking.
As he grows: your child's long-term outlook
Surgical techniques for treating Ebstein’s anomaly and its associated defects are continually being refined, and the long-term outlook is continually improving. Most children who’ve had surgery recover and grow normally.
Even so, your child will need periodic monitoring—and possibly medication—indefinitely, since he could be at some risk for abnormal heart rhythms (arrhythmias) and heart failure. In relatively few cases, patients who have had surgery for Ebstein’s anomaly may need additional surgery.
Your child’s cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who have had congenital heart disease repair will have an ongoing relationship with their cardiologist.
We’ll prevent and treat any complications and will advise on daily-life issues, such as activity levels, nutrition and precautions related to pregnancy.
Coping and support
We understand that if your child is has Ebstein’s anomaly, you’ll have a lot of questions. How serious is this condition? Will surgery help my child? What’s the long-term outlook? Children’s Hospital Boston has a variety of resources at that may be helpful to your family:
patient education: Our nurses will be on hand to walk you through your child’s treatment and help answer any questions you may have.
parent-to-parent: Want to talk with someone whose child has been treated for Ebstein’s anomaly? We can often put you in touch with other families who’ve been down a similar road and can share their experiences. To request family-to-family support, talk with your child’s health care team or contact The Center for Families.
faith-based support: If you and your family are in need of spiritual support, we can connect you with the Children’s chaplaincy. Our program includes nearly a dozen clergy representing Protestant, Jewish, Muslim, Roman Catholic and other faith traditions who will listen to you, pray with you and help you observe your own faith practices during your treatment experience.
social work and mental health professionals: Children’s social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with a wide variety of issues, such as coping with your child’s diagnosis, stresses relating to coping with illness and dealing with financial difficulties.
- As your child reaches adulthood, you’ll want him to know about our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH). Children’s is a founding institution of BACH, an international center for excellence providing long-term inpatient and outpatient care and advanced therapeutic options as needed for congenital heart disease patients as they reach and progress through adulthood.
|Children’s Development of Interventional Catheterization|
|You’ll be comforted to know that Children’s pioneered the use of interventional catheterization for many congenital heart defects and is a leader in the use of this procedure.|
|Keep family and friends up to date during your child’s treatment ...|
|… by creating a free Children’s Carepage.|
|Children’s Heart Care Center|
|The Heart Care Center at Children’s is one of the largest pediatric heart programs in the United States. Our staff of more than 80 pediatric cardiac specialists cares for thousands of children and adults with congenital and acquired heart defects each year, from simple to complex cases. We have experience treating rare heart problems—with results that are among the best in the world.|