Ebstein's Anomaly

Heart With Ebstein's Anomaly

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Ebstein's Anomaly is a rare defect, accounting for less than 1 percent of all congenital heart defects. The principal aberration occurring with Ebstein's Anomaly is a malformation of the tricuspid valve, which is located between the right atrium and right ventricle. Two of the three leaflets of the valve (the septal leaflet and the posterior leaflet) are displaced downward into the right ventricular cavity. These valve leaflets vary from mildly deformed to severely deformed. The third leaflet (the anterior leaflet) is not displaced but is typically large and redundant, often described as "sail-like". The portion of the right ventricle that sits above the displaced leaflets is usually thinner than normal and may be referred to as the atrialized portion of the right ventricle. A hole between the upper chambers of the heart, either an atrial septal defect or a patent foramen ovale, is virtually always present in association with Ebstein's anomaly. In some patients with this malformation, the pulmonary valve is also abnormal, either abnormally tight (pulmonary valve stenosis) or entirely closed (pulmonary valve atresia).

Most commonly, the deformed tricuspid valve has a tendency to leak, thus, as the right ventricle contracts some blood flows backwards from the right ventricle to the right atrium. Because of this backwards leakage of blood, a reduced volume of blood enters the right ventricle to be ejected to the lungs. The right atrium, which receives this leaking blood, is usually quite enlarged. Some of the blue blood from the right atrium may pass across the hole in the atrial septum into the left atrium. This blue blood then goes directly out to the body, which may be recognized as cyanosis in the patient. This is frequently present in newborns with Ebstein's anomaly and usually improves over the first weeks of life.

Infrequently, the deformed tricuspid valve forms an imperforate membrane. In this setting, rather than leaking, the tricuspid valve blocks blood from advancing into the right ventricle and out to the lungs.

In patients where the degree of valve deformity is mild and no symptoms are present, no intervention may be required other than prescribing antibiotics prior to dental or surgical procedures to prevent bacterial endocarditis. In patients with more severe involvement, cyanosis, shortness of breath, exercise intolerance and/or significant heart enlargement are often present and surgical intervention is warranted. The surgery usually consists of repairing or replacing the abnormal tricuspid valve and closing the hole between the atria.

What Is Normal Cardiac Anatomy?

When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

Learn More About Normal Cardiac Anatomy

Heart With Normal Cardiac Anatomy

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When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

The heart is composed of four chambers. The two upper chambers, known as atria, collect blood as it flows back to the heart. The two lower chambers, known as ventricles, pump blood with each heartbeat to the two main arteries (the pulmonary artery and the aorta). The septum is the wall that divides the heart into right and left sides. The atrial septum separates the right and left atria; likewise, the ventricular septum separates the two ventricles.

There are four valves that control the flow of blood through the heart. These flap-like structures allow blood to flow in only one direction. The tricuspid and mitral valves, also known as the atrioventricular valves, separate the upper and lower chambers of the heart. The aortic and pulmonary valves, also known as the arterial valves, separate the ventricles from the main arteries. Oxygen-depleted blood returns from the body and drains into the right atrium via the superior and inferior vena cavas. The blood in the right atrium then passes through the tricuspid valve and enters the right ventricle.

Next, the blood passes through the pulmonary valve, enters the pulmonary artery, and travels to the lungs where it is replenished with oxygen. The oxygen-rich blood returns to the heart via the pulmonary veins, draining into the left atrium. The blood in the left atrium passes through the bicuspid, or mitral, valve and enters the left ventricle.

Finally, the oxygen-rich blood flows through the aortic valve into the aorta and out to the rest of the body.

Congenital Heart Defects Special Needs Factsheet

What Teachers Should Know

Congenital heart defects develop in fetuses early in pregnancy, causing one abnormality or a combination of abnormalities in the structure of the heart. Some of the more common defects are:

  • holes in the walls between the heart's chambers
  • abnormal valves
  • abnormalities in the blood vessels entering or leaving the heart

About 8 out of every 1,000 newborns have congenital heart defects, which can range from mild to severe. Some may cause serious symptoms at birth, requiring intensive hospital care. Other defects may go undiagnosed until the teen or even adult years.

Most kids and teens with simple heart defects don't require any special care at school. Those with more complicated heart defects, however, may show signs that require attention, such as:

  • rapid breathing or shortness of breath
  • bluish coloring of the skin (cyanosis)
  • chest discomfort

Students with congenital heart defects may:

  • tire easily or want to avoid activities that require a lot of exertion
  • have activity restrictions
  • need to carry a water bottle to stay hydrated or stay indoors on hot days
  • need extra time to go to and from classes
  • need to go the bathroom a lot if they're taking diuretic medication
  • have bluish skin coloring due to low oxygen levels
  • need to use supplemental oxygen
  • need to go to the school nurse for medications
  • miss class time to see cardiologists or other medical professionals
  • have developmental delays or learning problems

What Teachers Can Do

Check with your student's parents or guardians to learn about the nature of the heart condition and any effects on learning and school activities.

If a student with a congenital heart defect misses class time for doctor's visits or hospitalizations, allow extra time for assignments and provide make-up work if appropriate. Most kids and teens with congenital heart defects can fully participate in most physical and extracurricular activities and should be encouraged to do so.

Reviewed by: Mary L. Gavin, MD
Date reviewed: September 26, 2016