Conditions + Treatments

Ebstein's anomaly in children

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"Parents have peace of mind knowing that Boston Children's uses the most innovative technologies and therapies for their child."

--David Harrild, MD, PhD, assistant in Cardiology 

Ebsteins Anomaly

If your infant or child has been diagnosed with Ebstein’s anomaly, an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, Ebstein’s anomaly is treatable surgically at Children’s Hospital Boston, and the outlook for many babies with this cardiac defect is good.

  • Ebstein’s anomaly is a rare heart defect of the tricuspid valve —affecting just one in 10,000 babies, and affecting boys and girls equally.
  • Ebstein’s anomaly has a wide range of severity—from mild to moderate to severe.
  • When Ebstein’s anomaly occurs, there are problems with the tricuspid valve. The valve is malformed and is positioned too low, allowing blood to leak backward from the ventricle to the atrium.
  • These abnormalities cause enlargement of the atrium and the “atrialization” of the right ventricle, leading to:
    • congestive heart failure—a back-up of blood flow that results in a fluid buildup in the lungs
    • insufficient red blood flow to the body (“blue baby” syndrome)
  • In addition, an opening in the wall between the two atria that occurs normally in the fetus sometimes fails to close.
    • This leads to a mixing of oxygen-rich and oxygen-poor blood, which results in poor oxygen levels in the blood that circulates through the baby’s body.
  • Ebstein’s anomaly often occurs with other heart defects, including atrial septal defect (ASD) and/or pulmonary (valve) stenosis (PVS, PS).
  • Associated arrhythmias and heart rate disturbances may also need treatment, usually by medications and/or catheter ablation.

How Boston Children's Hospital approaches Ebstein’s anomaly

Our team in Boston Children’s Cardiac Surgery Program understands how distressing a diagnosis of Ebstein’s anomaly can be for parents. You can have peace of mind knowing that our surgeons treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation among large pediatric cardiac centers. We use the following elements to provide the best possible outcomes:

  • accurate diagnosis and assessment: Subtle variations in heart anatomy—such as the arrangement of the arteries that feed the heart—can negatively impact surgical outcomes if not identified ahead of time. We utilize the most advanced techniques available for precisely determining your child’s heart anatomy, with interpretation by highly experienced cardiologists.
  • sophisticated and effective therapies: Babies with the most severe cases of Ebstein’s anomaly need emergency therapy. Our cardiac intensive care unit (CICU), cardiac cath labs and operating rooms deal with the urgent needs of our smallest patients 24/7. Children’s CICU was one of the first such units developed anywhere; professionals from many countries visit our CICU to learn advanced techniques of post-operative care.

    Boston Children’s Congenital Heart Valve Program cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques. 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. (See the Patient story in this topic.)
  • experienced team of skilled professionals to perform surgery and other procedures: Boston Children’s cardiac surgeons have vast experience in the surgical procedures used to repair this defect, and they work with nurses and doctors who are focused on providing expert care after surgery.
  • close, expert medical follow-up: If you live in the Boston area, a Boston Children’s cardiologist will follow your child after surgical repair; if you live in another part of the country or the world, a Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with Ebstein’s anomaly are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.

Ebstein’s anomaly: Reviewed by David Harrild, MD
© Boston Children's Hospital, 2010

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