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There are many ways you can help children and their families get the care they need.
It’s natural for you to be concerned right now about your child’s health—a diagnosis of Ebstein’s anomaly can be overwhelming. But you can rest assured that at Boston Children's Hospital, your child is in expert hands.
Our cardiologists, cardiac surgeons, cardiovascular imaging professionals and other clinicians have extensive experience with all types of heart problems. Each year, we treat thousands of children, adolescents and even adults with a wide variety of heart defects and disorders.
In a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, and is pumped by the right ventricle through the pulmonary artery into the lungs. In the lungs the blood receives oxygen (and gives up carbon dioxide), then returns to the left side of the heart. The left ventricle pumps the oxygen-rich blood through the aorta out to the body.
In a normal heart, the tricuspid valve controls blood flow from the right atrium, the chamber that receives blood returning from the body, to the right ventricle, the pumping chamber that pumps that blood to the lungs for oxygenation.
But in Ebstein’s anomaly, the leaflets that make up the tricuspid valve are malformed and are malpositioned too far down into the right ventricle. Sometimes, one of the leaflets is displaced downward into the ventricle, while another leaflet is larger than usual and may be abnormally attached to the wall of the ventricle.
When this occurs, blood leaks backward from the ventricle to the atrium. This causes enlargement of the atrium, and it can lead to congestive heart failure—a back-up of blood flow that results in a fluid buildup in the body.
In addition, an opening in the septum between the two atria that occurs normally in the fetus, called the foramen ovale, fails to close in some newborns with Ebstein's anomaly because of the high volume of blood in the right atrium. The opening, called the patent foramen ovale (PFO), allows the mixing of oxygen-poor blue blood from the right atrium with oxygen-rich red blood in the left atrium. This results in poor oxygen levels in the blood that circulates through the body.
There’s a very wide spectrum of variation and severity with Ebstein’s anomaly. The most severe cases are among the most challenging forms of heart disease—while the least severe forms are never even diagnosed, and children develop and function perfectly normally.
The severe forms of the defect cause such great enlargement of the heart—even prenatally—that the heart fills the baby’s entire chest cavity and crowds out the lungs, which end up being far too small. So, extreme cases end up not being just a heart problem, but rather, a heart-lung problem. The prognosis for such cases isn’t favorable, since by the time the baby is born, there’s already irreversible lung damage.
The good news is that such severe cases are quite uncommon. Far more common are milder forms of the disease, which are treatable, with usually favorable prognoses.
In Ebstein's anomaly, the tricuspid valve is downwardly displaced and adheres to the interventricular septum. Part of the right ventricle is “atrialized,” being located above the tricuspid valve. (abbreviations: Ao=aorta, LA=left atrium, LV=left ventricle, PA=pulmonary artery, RA=right atrium)
Often, Ebstein’s anomaly occurs with one or more other heart defects, including:
Arrhythmias can occur, too. Patients who have Ebstein’s anomaly may also have arrhythmias and abnormal heart rates that are associated with the condition. These may need treatment—usually by medications and/or catheter ablation (targeted destruction of affected tissue).
In 1990, Boston 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, Boston Children’s experts can call on our more than three decades’ worth of experience with this procedure.
While relatively rare (one in every 10,000 babies), and it affects boys and girls equally.
Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most of our patients who’ve had congenital heart disease have an ongoing relationship with their Children’s cardiologist.
We’ll prevent and treat complications, and will advise on daily-life issues, such as exercise and activity levels, nutrition and precautions related to pregnancy. Throughout his childhood, you can help your child—and the whole family—by making sure he eats a heart-healthy diet, exercises regularly, and in the teen years avoids smoking.
Surgical techniques for treating congenital heart defects are continually being refined, and Boston Children’s surgical success rates are very high. Nevertheless, your child will need periodic monitoring—and perhaps medication—indefinitely, since he could be at some risk for:
In relatively few cases, patients who’ve had surgery for Ebstein’s anomaly may need additional surgery.
As a congenital heart defect, Ebstein’s anomaly is a problem with your child’s heart structure that he was born with.
The heart forms during the first eight weeks of fetal development. It isn’t clear what causes congenital heart malformations, including Ebstein’s anomaly, although research is underway to search for a genetic or environmental link.
Cyanosis (blue coloration of the skin) is the most common sign of Ebstein’s anomaly in newborns and infants.
Call your health care provider immediately if your baby has a bluish color, or if your child is having difficulty breathing, is breathing rapidly, or seems to tire too easily.
Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported, but no genetic link has been confirmed. Research is underway to search for a genetic or environmental link. In general, if you have a child with a congenital cardiac defect, the chance of more children having a defect is about 2 to 3 percent.
If you’re a teen with a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you’ll also have to deal with medical appointments and procedures, some delay of your natural wish for independence, feeling different and assuming a lot of personal responsibility for maintaining your own good health.
If you feel overwhelmed, depressed or anxious through this important time in your transition to adulthood, speak to your doctor or counselor to get help.
If you were treated for congenital heart disease as a child, you’re probably being followed by your cardiologist, since complications from early heart disease can arise in adulthood. In particular, if you had surgery for Ebstein’s anomaly, you need lifelong monitoring—and possibly medication—since you’ll continue to be at some risk for heart problems in the future.
Fortunately, Boston Children’s can help adults with congenital heart defects. Many adults who were patients at Boston Children’s as babies or children continue to be monitored by the clinicians who have followed them since childhood.
In addition, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.
BACH is an international center for excellence, with physicians and services from Boston Children’s, Brigham and Women’s Hospital and the Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.
After surgery, your child’s cardiologist will offer recommendations for follow-up care, including:
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 he eats a heart-healthy diet, exercises regularly, and in the teen years avoids smoking.
For a more complete list of cardiovascular terms, visit our Cardiovascular glossary.
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We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”