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.

Heart Murmurs

What Are Heart Murmurs?

The human heartbeat is usually steady: lub-dub, lub-dub. In some people, though, the blood makes an extra noise as it flows through the heart. This sound is called a murmur.

Doctors hear a heart murmur as a whooshing sound between heartbeats. The whoosh is just an extra noise that the blood makes as it flows through the heart.

What Happens in a Heart Murmur?

Depending on a person's age, the heart beats about 60 to 120 times every minute. Each heartbeat is really two separate sounds. The heart goes "lub" with the closing of the valves that control blood flow from the upper chambers to the lower chambers. Then, as the valves controlling blood going out of the heart close, the heart goes "dub."

A heart murmur describes an extra sound heard in addition to the "lub-dub." Sometimes these extra sounds are simply the sound of normal blood flow moving through a normal heart. Other times, a murmur may be a sign of a heart problem.

Who Gets Heart Murmurs?

Parents might worry if they're told that their child has a heart murmur. But heart murmurs are very common, and many kids are found to have one at some point. Most murmurs are not a cause for concern and won't affect a child's health at all.

How Are Heart Murmurs Diagnosed?

Doctors listen to the heart by putting a stethoscope on different areas of the chest. It helps if kids are quiet as the doctor listens, because some heart murmurs are very soft. It's not unusual for a murmur to be noticed during a routine checkup, even if none was heard before.

Heart murmurs are rated on a scale from 1 to 6 based on how loud they are. Grade 1 is very soft, whereas grade 6 is very loud. If a murmur is found, the doctor may refer a child to a pediatric cardiologist for further evaluation.

What Causes Heart Murmurs?

Innocent Heart Murmurs

The most common type of heart murmur is called functional or innocent. An innocent heart murmur is the sound of blood moving through a normal, healthy heart in a normal way. Just as you might hear air moving through an air duct or water flowing through a pipe, doctors can hear blood moving through the heart even when there's no heart problem.

An innocent heart murmur can come and go throughout childhood. Kids with these murmurs don't need a special diet, restriction of activities, or any other special treatment. Those old enough to understand that they have a heart murmur should be reassured that they aren't any different from other kids.

Most innocent murmurs will go away on their own as a child gets older.

Congenital Heart Defects

Some murmurs can indicate a problem with the heart. In these cases, doctors will have a child see a pediatric cardiologist. The cardiologist will order tests such as:

  • a chest X-ray: a picture of the heart and surrounding organs
  • an EKG: a record of the heart's electrical activity
  • an echocardiogram: a picture of the heart made using sound waves

About 1 out of every 100 babies is born with a structural heart problem, or congenital heart defect. These babies may show signs of the defect as early as the first few days of life or not until later in childhood. Some kids won't have any symptoms beyond a heart murmur, while others will such signs as:

  • rapid breathing
  • difficulty feeding
  • blueness in the lips (called cyanosis)
  • failure to thrive

An older child or teen might:

  • be very tired
  • have trouble exercising or doing physical activity
  • have chest pain

Call your doctor if your child has any of these symptoms.

Pregnant women have a higher risk of having a baby with a heart defect if they get rubella (German measles), have poorly controlled diabetes, or have PKU (phenylketonuria, a genetic error of the body's metabolism).

Common Heart Defects

Several kinds of heart problems can cause heart murmurs, including:

  • Septal defects, which involve the walls (or septum) between the upper or lower chambers of the heart. A hole in the septum can let blood flow through it into the heart's other chambers. This extra blood flow may cause a murmur. It can also make the heart work too hard or become enlarged. Bigger holes can cause symptoms besides a heart murmur; smaller ones may eventually close on their own.
  • Valve abnormalities, caused by heart valves that are narrow, too small, too thick, or otherwise abnormal. These valves don't allow smooth blood flow across them. Sometimes, they can allow backflow of blood within the heart. Either problem will cause a murmur. Outflow tract obstruction might be caused by extra tissue or heart muscle that blocks the smooth flow of blood through the heart.
  • Heart muscle disorders (cardiomyopathy), which can make the heart muscle abnormally thick or weak, hurting its ability to pump blood to the body normally.

Your doctor and a pediatric cardiologist can determine if the murmur is innocent (which means your child is perfectly healthy) or if there is a specific heart problem. If there is a problem, the pediatric cardiologist will know how best to take care of it.

Reviewed by: Steven B. Ritz, MD
Date reviewed: September 05, 2017